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Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report
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ANNPLA-1452; No. of Pages 8 Annales de chirurgie plastique esthétique (2018) xxx, xxx—xxx
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CLINICAL CASE
Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report ´ e par Sarcome `a cellules claires de la langue mobile traite glossectomie subtotale et reconstruction par lambeau libre DIEP : ´ rature `a propos d’un cas et revue de litte A. Baus a,*, D. Culie b, L.T. Duong b, A. Ben Lakhdar c, J.-B. Schaaf a, F. Janot b, F. Kolb a Institut Gustave-Roussy, department of plastic surgery, Gustave-Roussy, 114 rue ´Edouard-Vaillant, 94805 Villejuif, France b Institut Gustave-Roussy, department of head and neck oncology, 114, rue ´Edouard-Vaillant, 94805 Villejuif, France c Institut Gustave-Roussy, department of biopathology, 114, rue ´Edouard-Vaillant, 94805 Villejuif, France a
Received 31 July 2018; accepted 3 September 2018
KEYWORDS Clear cell sarcoma; Oral tongue glossectomy; Head and neck reconstruction; DIEP free flap; ‘‘Cathedral triptych’’ design
Summary Clear cell sarcomas (SCC), also called ‘‘soft-tissue melanoma’’, are rare and aggressive tumors that preferentially affect the lower limbs (tendons and fasciae) and which have also been described in head and neck localizations. Their clinical and immunohistochemical mimicry with melanoma makes it difficult to diagnose sarcomas. SCC treatment is mainly focused on large-scale resection surgery with adjuvant radiotherapy because of their low chemosensitivity and extreme lymphophilia. In case of head and neck localization, these treatments may lead to function and aesthetic sequelae thus requiring the use of modern techniques of reconstructive surgery. The authors describe the diagnosis, treatment and follow-up of large lingual SCC case using a DIEP free flap reconstruction according to an original technique developed in the department. Given the characteristics of patients with SCC (a high proportion of women between 20 and 40 years old) and its inherent qualities (low morbidity of the donor site, volume delivered and excellent plasticity), the fascio-cutaneous free flap type ‘‘DIEP’’
* Corresponding author. Institut Gustave-Roussy, department of plastic surgery, Gustave-Roussy, 114 rue Édouard-Vaillant, 94805 Villejuif, France. E-mail address: [email protected] (A. Baus). https://doi.org/10.1016/j.anplas.2018.09.001 0294-1260/# 2018 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Baus A, et al. Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report . Ann Chir Plast Esthet (2018), https://doi.org/10.1016/j.anplas.2018.09.001
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A. Baus et al. ‘‘taken according to the design of the’’ Cathedral triptych seems to be a viable choice among the range of reconstruction solutions. # 2018 Elsevier Masson SAS. All rights reserved.
MOTS CLÉS Sarcome à cellules claires ; Glossectomie subtotale de langue mobile ; Reconstruction tête et cou ; Lambeau libre DIEP ; Design du « Triptyque de cathédrale »
Re ´sume ´ Les sarcomes à cellules claires (SCC), également baptisées « mélanomes des parties molles » sont des tumeurs rares et agressives affectant préférentiellement les membres inférieurs (tendons et aponévroses), mais pouvant s’observer dans des localisations ORL. De part leur mimétisme clinique et immunohistochimique avec le mélanome, les sarcomes restent encore aujourd’hui de diagnostic difficile. Compte tenu de leur faible chimiosensibilité et de leur extrême lymphophilie, le traitement des SCC est essentiellement axé sur la chirurgie d’exérèse large associé à de la radiothérapie adjuvante. En cas de localisation ORL, ces traitements peuvent être responsable de pertes de substances délabrantes sur le plan fonctionnel et esthétique, nécessitant l’utilisation de techniques modernes de chirurgie réparatrice. Les auteurs décrivent le diagnostic, le traitement et le suivi d’un cas de volumineux SSC lingual ayant fait appel à une reconstruction par lambeau libre DIEP selon une technique originale développée dans le service. Compte tenu des caractéristiques des patients atteints de SCC (proportion importante de femmes entre 20 et 40 ans), et de ses qualités propres (faible morbidité du site donneur, volume apporté et excellente plasticité), le lambeau libre fascio-cutané de type « DIEP » prélevé selon le dessin du « tryptique de la cathédrale » semble tenir une place de choix parmi l’éventail des solutions de reconstruction. # 2018 Elsevier Masson SAS. Tous droits réservés.
Introduction Clear cell sarcomas (CCS) are rare soft-tissue tumors that preferentially affect tendons and fascia of distal extremities [1]. They were discovered and published for the first time in the literature by Enzinger [2]. These CCS can mimic an inflammatory granuloma of subcutaneous tissues, preferentially affecting young adults (median age: 27 years) [3] with a female predominance [1,2,4—6]. Since its first description in 1970, this specific type of sarcoma has been described in other localizations, such as pure dermal lesions [7], the gastrointestinal tract [8] or the oral cavity [9]. Considering their extreme lymphophily [10,11] and the high frequency of locoregional recurrences with deep extension and late metastases [12], surgical excision must be widened, taking away the peri-lesional lymph networks. Follow-up must also be prolonged [13]. Current therapeutic management that consists of an oncologic surgery with or without adjuvant chemotherapy allows a 5-year survival rate of approximately 50 to 65%. The head and neck localizations of CCS are characterized by invasive surgeries that are responsible for significant functional and aesthetic sequelae [14]. Despite the progress of reconstructive surgery such as the advent of innervated perforating flaps [15] and new flap designs to reproduce the shapes and reliefs of the oral cavity [16,17], lingual reconstruction still remains nowadays a real challenge. The authors describe a case of CCS of the mobile tongue that required a total glossectomy with lingual reconstruction using a deep inferior epigastric perforator (DIEP) free flap according to the design of the ‘‘cathedral triptych’’ [16].
Case presentation We present the case of a 44-year-old patient with no medical history except 2 pregnancies, without any cigarette smoking
and alcohol intoxication. She was referred to our institution for the management of a CCS of the tongue. Lingual discomfort and mild dysphagia to solid food progressively worsened starting from March 2017. Clinical examination objectified a correct tongue protraction with a welllimited and hard tumefaction on the ventral surface of the tongue. There was no mucosal disease and no suspicious cervical lymphadenopathies noticed at the clinical exam.
Oncologic management Locoregional extension of the tumor was assessed with an MRI exam (Fig. 1A, B, and C). MRI showed an isolated welldefined mass of the oral tongue, measuring 41 36 38 mm with no invasion of the neurovascular bundles of/located in the base of the tongue. A PET-scan was realized for the extension work-up (Fig. 1D). No other lesion was identified, neither pathologic cervical lymph nodes. The PET scan confirmed the presence of a hypermetabolic and isolated lesion of the tongue. Deep biopsies were realized during a panendoscopy under general anesthesia. First histologic report found a malignant proliferation of clears cells, with a lobule organization separated by collagene fibrosis bays. Immunohistochemical analysis was positive for P100 and Bcl2 markers, CK7, CD34, Synaptophysine and CD 56 was weakly and heterogenous positives, CKAE1/AE3, Chromogranin A, HMB45, AML, Desmine, p63, CD68 and EMA was negatives. Ki67 proliferation index was 10 to 20%. At this step, two entities were considered: clear cell sarcoma and a malignant melanoma. Further analysis was realized in a tertiary cancer center (Curie Institute), a specific translocation of the EWTS1 gene, specific translocation of clear cell sarcoma, and an amplification of the BAP-1 gene without other ‘‘activated’’ mutations (BRAF and NRAS not mutated) were found. The tumors cells did not express PDL-1.
Please cite this article in press as: Baus A, et al. Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report . Ann Chir Plast Esthet (2018), https://doi.org/10.1016/j.anplas.2018.09.001
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Figure 1 A. T1 MRI ‘‘Fat Sat’’ with gadolinium. Respect of the vascular pedicles of the tongue’s base (white arrow). B. T2 MRI (sagittal section). C. T1 MRI, transverse section, lesion centered on the oral tongue. D. Axial fused PET/CT section.
This tumor was such classified as a T3N0M0 with the 8th TNM classification of the UICC/AJCC. The therapeutic decision was first discussed in the French Network of rare head and neck tumors (REFCOR). It was proposed to realize first a neo-adjuvant chemotherapy (5PLC-AI cycles: Adriamycine, Cisplatinium, Ifosfamide). No response was observed after chemotherapy, with a 13% progression observed by MRI exam. National network and local tumor board such proposed a surgical treatment (described below). Histologic analysis of the tumor after surgery confirmed the diagnosis of clear cell sarcoma, with a 50 mm tumor size, negative margins but inferior to 1 mm on the posterior side, without lymph nodes involvement. Local tumor board, as REFCOR, recommended realizing adjuvant radiotherapy.
Surgical technique After the inefficiency of the neo-ajvuvant chemotherapy protocol, a sub-total transversal glossectomy of the oral tongue was indicated. A pull-through procedure combined
with bilateral modified radical cervical dissection and lingual reconstruction was performed at the same time. The choice of reconstruction was based on the following criteria’s: the patient’s morphotype: long-limbed with a thin adipose panicle at the thighs, and a slight cutaneous-fatty excess under umbilical after her two pregnancies; the sacrifice of almost all of the oral tongue involves the choice of a fascio-cutaneous flap, whose volume and plasticity are sufficient to form the flap with the ‘‘triptych of the cathedral’’; the loss of a part of the buccal floor musculature, added to the pull-through procedure of the tumor removal, that involves a composite fascio-cutaneous flap including a fascial band which allows to reconstruct the hyoid strap and to obtain a laryngeal suspension effect. An anterolateral thigh flap (‘‘ALT Flap’’) with a part of fascia lata or fascio-cutaneous flaps of ‘‘DIEP’’ with a fascial band of the rectus abdominis muscle were considered.
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The patient was informed about the different morbidity and the esthetic sequelae of the different donor sites. We decided to perform a DIEP free flap including a fascial band of the rectus abdominis muscle.
the mobile tongue could be place under the mandible to guarantee the posterior control of the tumor near the base of tongue, and the tumor has been resected.
Lymph nodes and tumor removal step
Harvesting of the flap and microvascular anastomosis
Bilateral cervical lymph node was first realized with the habitual technique. Then, tumor removal began with a trans-oral approach by the anterior section of the anterior buccal floor of the mucosa. The buccal floor musculature was liberated from its mandibular insertion. With the pull-through procedure,
The first step consisted in a preoperative echographic localization of the main peri-umbilical perforating vessels (see Fig. 2A) and the simple outline of the cutaneous palette. The DIEP flap was raised by incising the lower part of the cutaneous palette followed by a dissection of the rectus abdominis’ fascia until the main perforating vessels were found.
Figure 2 A. Doppler detection of the main cutaneous perforators. B/C. Preparatory drawing/pattern ‘‘Triptych of the Cathedral’’. D. Flap centered on cutaneous perforators. E. Flap on its vascular pedicle. F. Flap’s conformation on its donor site. Please cite this article in press as: Baus A, et al. Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report . Ann Chir Plast Esthet (2018), https://doi.org/10.1016/j.anplas.2018.09.001
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Primary clear cell sarcoma of the oral tongue The definitive peroperative drawing/pattern of the flap was centered on the perforators which appeared to be the most reliable (Fig. 2D). Dissection of the pedicle was continued until its origin (external iliac artery). A rectus abdominis muscle fascia band was removed during the dissection and the loss of substance was closed with an overlock suture. Once a vascular island flap was achieved (Fig. 2E), the cutaneous palette was shaped in anticipation of weaning and microsurgical transfer (Fig. 2F). The vascular anastomoses were performed on the superior thyroid artery, then the flap was positioned and sutured in the mouth on the lingual stump after reinforcement of the suprahyoid muscles via the removed fascia.
Post-surgery follow-up There were no significant postoperative complications. The patient was discharged to a rehabilitation unit at day 20 and decanulation occurred at D26. Complete excision of the lesion (diameter: 5 cm) was achieved with a posterior margin of only 1 mm. There was no peri-neural sheathing or vascular embolism. The cutoffs made at the floor of the mouth were cancer-free as well as the lymph nodes removed (24 negative lymph nodes out of 24 analyzed for the right side, 25 negative lymph nodes out of 25 analyzed for the left side, group IA: 2 negative out of 2 analyzed) (Fig. 3). Radiotherapy with irradiation of the tumor bed and of the posterior portion of the flap was performed (total dose = 65 Gy).
5 Reeducation combining sessions of speech therapy with sessions of physiotherapy was undertaken. Removal of the nasogastric tube was performed 35 days after surgery and oral feeding was resumed (Fig. 3). However, a gastrostomy tube had to be placed during radiotherapy because of its side effects. At 6 months postoperative, The gastrostomy tube is now removed. The patient is able to eat mixed foods and express herself comprehensibly. Nevertheless, she continues her speech therapy and reeducation sessions.
Discussion CCSs are rare and aggressive tumors (1% of all types of sarcomas combined) affecting preferentially the tendon and fascia of the lower limbs (40% of SCC) [1,2,4—6]. Head and localizations are fortunately rare, and very few clinical cases are published in the literature [13,18—20]. Their clinical presentation is nonspecific, with a hard tumor of a variable volume, and 50 to 66% of patients with SCC report no pain and so did our patient [21]. CCSs are also known as ‘‘soft-tissue melanoma’’ because their clinical presentation can mimic cutaneous melanoma and because of the expression of melanocyte markers in immunohistochemistry (S100, HMB-45, MITF, etc.). They are characterized by a translocation t [12,22] (q13; q12), absent in melanomas, which allows their identification in 90% of the cases [19,20,22,23]. CCS’ diagnosis of certainty, particularly at the metastatic stage, remains problematic for the anatomopathologist [24].
Figure 3 A. Operative piece: grossing specimen of subtotal transversal glossectomy. B. Lobulated and well defined lesion within tong’s wall. C. Epithelioid clear cell proliferation presented alveolar structure. Cells lobules separated by collagen fibrosis bay ( 200). D. Lobulated and well defined tumor with stroma’s fibro-oedmatous change due to neo-adjuvant chemotherapy ( 5). Please cite this article in press as: Baus A, et al. Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report . Ann Chir Plast Esthet (2018), https://doi.org/10.1016/j.anplas.2018.09.001
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A number of biomarkers seem to have been effective in ruling out nodular melanoma [7,25,26]. This main differential diagnosis has a high metastatic risk and requires different treatment modalities than those of CCS [3]. There are few therapeutic guidelines for head and neck soft tissues sarcoma management in adults, surgery remains the gold standard. A monoblock resection with negative margins must be realized. There is no consensus to determine margin width, 2 cm is generally acceptable [27]. In this case, the posterior margin was inferior. Nevertheless, 2 cm posterior margin would have removed the totality of the base of tongue, with such an impossibility to recover an oral alimentation, a high risk of secondary functional laryngectomy. Most authors do not recommend lymph nodes surgery because regional lymph nodes metastases are rare [28]. However, two entities are exceptions: epithelioid sarcoma and clear cell sarcoma that is why we performed a bilateral neck dissection [29]. Bones and lungs are the other common sites of metastatic recurrence [12,30—34]. If resection margins are too close (R0/R1), radiotherapy is indicated since it has shown better local control of the tumor [4,35,36]. Neo-adjuvant chemotherapy for head and neck sarcoma is commonly realized in case of advanced inoperable tumor, or for tumor regression to achieve a R0/R1 resection or to limit a mutilating surgery [37]. However, certain histologic subtypes as soft-tissue alveolar sarcoma, well differentied liposarcoma and clear-cell sarcoma are generally less responsive to chemotherapy [27,38]. The lesion’s size is also of importance as it determines the adjuvant therapeutic strategy for surgery, for instance the initiation of chemotherapy for tumors larger than 5 cm in diameter [39,40]. In our case, the non-response to neoadjuvant chemotherapy lead to propose radiotherapy alone in postoperative care.
Figure 4
All these considerations lead to mutilating surgeries, particularly for head and neck sites, confirming the key role of reconstruction techniques. The main goals of reconstructive surgery following head and neck cancer are multiple. The volumes and the reliefs of the oral cavity have to be maintained. The dead spaces have to be filled. Moreover, functional and volumetric ‘‘ad integrum’’ recovery should always be sought after as well as limiting as much as possible postoperative complications. Tongue reconstructions are of utmost importance as it plays a social (speech, oral hygiene) but also nutritional (chewing, feeding) role. Overall improvement of the survival rate of patients with tongue cancer further reinforces the fact that postoperative quality of life has been to taken into account/ the importance of the concept of postoperative quality of life. Guidelines regarding the type of technique to choose depending on the loss of tongue substance have been proposed and are commonly used for tongue reconstruction and rehabilitation [41]. As such, many technical variants have been described. Free flaps, thanks to their plasticity, their ease of use, the low morbidity of the donor site as well as the possibility of providing well-vascularized composite tissues, play a key role. In 1989, Koshima and Soeda [15] were the first to use a fascio-cutaneous perforating DIEP flap in Head and neck reconstruction. Mostly used in breast reconstruction, this flap has the advantage of bringing a large volume with a low morbidity of the donor site. Based on these arguments, Yano et al. [42,43] therefore believe that the DIEP flap is the flap of choice in pediatric tongue reconstruction. However, compared to the musculo-cutaneous flaps, it should be noted that the cutaneous and adipose tissues are less vascularized which could increase postoperative complications like infections or postradiation oro-cutaneous fistulas [42]. Furthermore, loss of volume can occur over the time and be
A—B. Two-month postoperative flap appearance. C. Ventral surface of the flap. Note the limited lingual elevation.
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Two-month postoperative view of the donor site.
aggravated by radiotherapy [44]. DIEPs detractors believe that for Head and neck localizations, thinner and malleable flaps, such as the radial forearm flap [45,46] or the anterolateral thigh flap [16], with their possibility of re-innervation, should be favored for tongue reconstruction/have a preferential place in the indication of tongue reconstruction in the adult. According to our experience, oral tongue resections/ glossectomy give the worst functional results after total glossectomy. The objective of this reconstruction was thus threefold: to bring a sufficient volume to allow a good function (and to limit post radiation volume loss); to ensure sufficient plasticity and malleability for the conformation of the flap 3. To limit the aesthetic sequelae of the donor site by ensuring a direct closure (Fig. 5).
Conclusion CCSs are aggressive tumors whose head and neck localization is extremely rare. Their treatment remains mainly surgical. The main differential diagnosis that has to be ruled out is melanoma. Early management is primary since a broad excision followed by adjuvant therapies can cause many aesthetic and functional sequelae. Reconstructive surgery is therefore essential but still remains a challenge that requires to carefully choosing the most appropriate solution for the patient, as well as informing him of the expected results. Given the characteristics of patients suffering from CCS (women between 20 and 40 years), and CCS own characteristics (low morbidity of the donor site, adequate volume of the flap and excellent plasticity), the fasciocutaneous free flap ‘‘DIEP’’ taken according to the pattern
of the ‘‘triptych of the cathedral’’ seems to be a viable choice among the range of reconstruction solutions.
Disclosure of interest The authors declare that they have no competing interest.
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ANNPLA-1452; No. of Pages 8 Annales de chirurgie plastique esthétique (2018) xxx, xxx—xxx
Available online at
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CLINICAL CASE
Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report ´ e par Sarcome `a cellules claires de la langue mobile traite glossectomie subtotale et reconstruction par lambeau libre DIEP : ´ rature `a propos d’un cas et revue de litte A. Baus a,*, D. Culie b, L.T. Duong b, A. Ben Lakhdar c, J.-B. Schaaf a, F. Janot b, F. Kolb a Institut Gustave-Roussy, department of plastic surgery, Gustave-Roussy, 114 rue ´Edouard-Vaillant, 94805 Villejuif, France b Institut Gustave-Roussy, department of head and neck oncology, 114, rue ´Edouard-Vaillant, 94805 Villejuif, France c Institut Gustave-Roussy, department of biopathology, 114, rue ´Edouard-Vaillant, 94805 Villejuif, France a
Received 31 July 2018; accepted 3 September 2018
KEYWORDS Clear cell sarcoma; Oral tongue glossectomy; Head and neck reconstruction; DIEP free flap; ‘‘Cathedral triptych’’ design
Summary Clear cell sarcomas (SCC), also called ‘‘soft-tissue melanoma’’, are rare and aggressive tumors that preferentially affect the lower limbs (tendons and fasciae) and which have also been described in head and neck localizations. Their clinical and immunohistochemical mimicry with melanoma makes it difficult to diagnose sarcomas. SCC treatment is mainly focused on large-scale resection surgery with adjuvant radiotherapy because of their low chemosensitivity and extreme lymphophilia. In case of head and neck localization, these treatments may lead to function and aesthetic sequelae thus requiring the use of modern techniques of reconstructive surgery. The authors describe the diagnosis, treatment and follow-up of large lingual SCC case using a DIEP free flap reconstruction according to an original technique developed in the department. Given the characteristics of patients with SCC (a high proportion of women between 20 and 40 years old) and its inherent qualities (low morbidity of the donor site, volume delivered and excellent plasticity), the fascio-cutaneous free flap type ‘‘DIEP’’
* Corresponding author. Institut Gustave-Roussy, department of plastic surgery, Gustave-Roussy, 114 rue Édouard-Vaillant, 94805 Villejuif, France. E-mail address: [email protected] (A. Baus). https://doi.org/10.1016/j.anplas.2018.09.001 0294-1260/# 2018 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Baus A, et al. Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report . Ann Chir Plast Esthet (2018), https://doi.org/10.1016/j.anplas.2018.09.001
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A. Baus et al. ‘‘taken according to the design of the’’ Cathedral triptych seems to be a viable choice among the range of reconstruction solutions. # 2018 Elsevier Masson SAS. All rights reserved.
MOTS CLÉS Sarcome à cellules claires ; Glossectomie subtotale de langue mobile ; Reconstruction tête et cou ; Lambeau libre DIEP ; Design du « Triptyque de cathédrale »
Re ´sume ´ Les sarcomes à cellules claires (SCC), également baptisées « mélanomes des parties molles » sont des tumeurs rares et agressives affectant préférentiellement les membres inférieurs (tendons et aponévroses), mais pouvant s’observer dans des localisations ORL. De part leur mimétisme clinique et immunohistochimique avec le mélanome, les sarcomes restent encore aujourd’hui de diagnostic difficile. Compte tenu de leur faible chimiosensibilité et de leur extrême lymphophilie, le traitement des SCC est essentiellement axé sur la chirurgie d’exérèse large associé à de la radiothérapie adjuvante. En cas de localisation ORL, ces traitements peuvent être responsable de pertes de substances délabrantes sur le plan fonctionnel et esthétique, nécessitant l’utilisation de techniques modernes de chirurgie réparatrice. Les auteurs décrivent le diagnostic, le traitement et le suivi d’un cas de volumineux SSC lingual ayant fait appel à une reconstruction par lambeau libre DIEP selon une technique originale développée dans le service. Compte tenu des caractéristiques des patients atteints de SCC (proportion importante de femmes entre 20 et 40 ans), et de ses qualités propres (faible morbidité du site donneur, volume apporté et excellente plasticité), le lambeau libre fascio-cutané de type « DIEP » prélevé selon le dessin du « tryptique de la cathédrale » semble tenir une place de choix parmi l’éventail des solutions de reconstruction. # 2018 Elsevier Masson SAS. Tous droits réservés.
Introduction Clear cell sarcomas (CCS) are rare soft-tissue tumors that preferentially affect tendons and fascia of distal extremities [1]. They were discovered and published for the first time in the literature by Enzinger [2]. These CCS can mimic an inflammatory granuloma of subcutaneous tissues, preferentially affecting young adults (median age: 27 years) [3] with a female predominance [1,2,4—6]. Since its first description in 1970, this specific type of sarcoma has been described in other localizations, such as pure dermal lesions [7], the gastrointestinal tract [8] or the oral cavity [9]. Considering their extreme lymphophily [10,11] and the high frequency of locoregional recurrences with deep extension and late metastases [12], surgical excision must be widened, taking away the peri-lesional lymph networks. Follow-up must also be prolonged [13]. Current therapeutic management that consists of an oncologic surgery with or without adjuvant chemotherapy allows a 5-year survival rate of approximately 50 to 65%. The head and neck localizations of CCS are characterized by invasive surgeries that are responsible for significant functional and aesthetic sequelae [14]. Despite the progress of reconstructive surgery such as the advent of innervated perforating flaps [15] and new flap designs to reproduce the shapes and reliefs of the oral cavity [16,17], lingual reconstruction still remains nowadays a real challenge. The authors describe a case of CCS of the mobile tongue that required a total glossectomy with lingual reconstruction using a deep inferior epigastric perforator (DIEP) free flap according to the design of the ‘‘cathedral triptych’’ [16].
Case presentation We present the case of a 44-year-old patient with no medical history except 2 pregnancies, without any cigarette smoking
and alcohol intoxication. She was referred to our institution for the management of a CCS of the tongue. Lingual discomfort and mild dysphagia to solid food progressively worsened starting from March 2017. Clinical examination objectified a correct tongue protraction with a welllimited and hard tumefaction on the ventral surface of the tongue. There was no mucosal disease and no suspicious cervical lymphadenopathies noticed at the clinical exam.
Oncologic management Locoregional extension of the tumor was assessed with an MRI exam (Fig. 1A, B, and C). MRI showed an isolated welldefined mass of the oral tongue, measuring 41 36 38 mm with no invasion of the neurovascular bundles of/located in the base of the tongue. A PET-scan was realized for the extension work-up (Fig. 1D). No other lesion was identified, neither pathologic cervical lymph nodes. The PET scan confirmed the presence of a hypermetabolic and isolated lesion of the tongue. Deep biopsies were realized during a panendoscopy under general anesthesia. First histologic report found a malignant proliferation of clears cells, with a lobule organization separated by collagene fibrosis bays. Immunohistochemical analysis was positive for P100 and Bcl2 markers, CK7, CD34, Synaptophysine and CD 56 was weakly and heterogenous positives, CKAE1/AE3, Chromogranin A, HMB45, AML, Desmine, p63, CD68 and EMA was negatives. Ki67 proliferation index was 10 to 20%. At this step, two entities were considered: clear cell sarcoma and a malignant melanoma. Further analysis was realized in a tertiary cancer center (Curie Institute), a specific translocation of the EWTS1 gene, specific translocation of clear cell sarcoma, and an amplification of the BAP-1 gene without other ‘‘activated’’ mutations (BRAF and NRAS not mutated) were found. The tumors cells did not express PDL-1.
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Figure 1 A. T1 MRI ‘‘Fat Sat’’ with gadolinium. Respect of the vascular pedicles of the tongue’s base (white arrow). B. T2 MRI (sagittal section). C. T1 MRI, transverse section, lesion centered on the oral tongue. D. Axial fused PET/CT section.
This tumor was such classified as a T3N0M0 with the 8th TNM classification of the UICC/AJCC. The therapeutic decision was first discussed in the French Network of rare head and neck tumors (REFCOR). It was proposed to realize first a neo-adjuvant chemotherapy (5PLC-AI cycles: Adriamycine, Cisplatinium, Ifosfamide). No response was observed after chemotherapy, with a 13% progression observed by MRI exam. National network and local tumor board such proposed a surgical treatment (described below). Histologic analysis of the tumor after surgery confirmed the diagnosis of clear cell sarcoma, with a 50 mm tumor size, negative margins but inferior to 1 mm on the posterior side, without lymph nodes involvement. Local tumor board, as REFCOR, recommended realizing adjuvant radiotherapy.
Surgical technique After the inefficiency of the neo-ajvuvant chemotherapy protocol, a sub-total transversal glossectomy of the oral tongue was indicated. A pull-through procedure combined
with bilateral modified radical cervical dissection and lingual reconstruction was performed at the same time. The choice of reconstruction was based on the following criteria’s: the patient’s morphotype: long-limbed with a thin adipose panicle at the thighs, and a slight cutaneous-fatty excess under umbilical after her two pregnancies; the sacrifice of almost all of the oral tongue involves the choice of a fascio-cutaneous flap, whose volume and plasticity are sufficient to form the flap with the ‘‘triptych of the cathedral’’; the loss of a part of the buccal floor musculature, added to the pull-through procedure of the tumor removal, that involves a composite fascio-cutaneous flap including a fascial band which allows to reconstruct the hyoid strap and to obtain a laryngeal suspension effect. An anterolateral thigh flap (‘‘ALT Flap’’) with a part of fascia lata or fascio-cutaneous flaps of ‘‘DIEP’’ with a fascial band of the rectus abdominis muscle were considered.
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The patient was informed about the different morbidity and the esthetic sequelae of the different donor sites. We decided to perform a DIEP free flap including a fascial band of the rectus abdominis muscle.
the mobile tongue could be place under the mandible to guarantee the posterior control of the tumor near the base of tongue, and the tumor has been resected.
Lymph nodes and tumor removal step
Harvesting of the flap and microvascular anastomosis
Bilateral cervical lymph node was first realized with the habitual technique. Then, tumor removal began with a trans-oral approach by the anterior section of the anterior buccal floor of the mucosa. The buccal floor musculature was liberated from its mandibular insertion. With the pull-through procedure,
The first step consisted in a preoperative echographic localization of the main peri-umbilical perforating vessels (see Fig. 2A) and the simple outline of the cutaneous palette. The DIEP flap was raised by incising the lower part of the cutaneous palette followed by a dissection of the rectus abdominis’ fascia until the main perforating vessels were found.
Figure 2 A. Doppler detection of the main cutaneous perforators. B/C. Preparatory drawing/pattern ‘‘Triptych of the Cathedral’’. D. Flap centered on cutaneous perforators. E. Flap on its vascular pedicle. F. Flap’s conformation on its donor site. Please cite this article in press as: Baus A, et al. Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report . Ann Chir Plast Esthet (2018), https://doi.org/10.1016/j.anplas.2018.09.001
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Primary clear cell sarcoma of the oral tongue The definitive peroperative drawing/pattern of the flap was centered on the perforators which appeared to be the most reliable (Fig. 2D). Dissection of the pedicle was continued until its origin (external iliac artery). A rectus abdominis muscle fascia band was removed during the dissection and the loss of substance was closed with an overlock suture. Once a vascular island flap was achieved (Fig. 2E), the cutaneous palette was shaped in anticipation of weaning and microsurgical transfer (Fig. 2F). The vascular anastomoses were performed on the superior thyroid artery, then the flap was positioned and sutured in the mouth on the lingual stump after reinforcement of the suprahyoid muscles via the removed fascia.
Post-surgery follow-up There were no significant postoperative complications. The patient was discharged to a rehabilitation unit at day 20 and decanulation occurred at D26. Complete excision of the lesion (diameter: 5 cm) was achieved with a posterior margin of only 1 mm. There was no peri-neural sheathing or vascular embolism. The cutoffs made at the floor of the mouth were cancer-free as well as the lymph nodes removed (24 negative lymph nodes out of 24 analyzed for the right side, 25 negative lymph nodes out of 25 analyzed for the left side, group IA: 2 negative out of 2 analyzed) (Fig. 3). Radiotherapy with irradiation of the tumor bed and of the posterior portion of the flap was performed (total dose = 65 Gy).
5 Reeducation combining sessions of speech therapy with sessions of physiotherapy was undertaken. Removal of the nasogastric tube was performed 35 days after surgery and oral feeding was resumed (Fig. 3). However, a gastrostomy tube had to be placed during radiotherapy because of its side effects. At 6 months postoperative, The gastrostomy tube is now removed. The patient is able to eat mixed foods and express herself comprehensibly. Nevertheless, she continues her speech therapy and reeducation sessions.
Discussion CCSs are rare and aggressive tumors (1% of all types of sarcomas combined) affecting preferentially the tendon and fascia of the lower limbs (40% of SCC) [1,2,4—6]. Head and localizations are fortunately rare, and very few clinical cases are published in the literature [13,18—20]. Their clinical presentation is nonspecific, with a hard tumor of a variable volume, and 50 to 66% of patients with SCC report no pain and so did our patient [21]. CCSs are also known as ‘‘soft-tissue melanoma’’ because their clinical presentation can mimic cutaneous melanoma and because of the expression of melanocyte markers in immunohistochemistry (S100, HMB-45, MITF, etc.). They are characterized by a translocation t [12,22] (q13; q12), absent in melanomas, which allows their identification in 90% of the cases [19,20,22,23]. CCS’ diagnosis of certainty, particularly at the metastatic stage, remains problematic for the anatomopathologist [24].
Figure 3 A. Operative piece: grossing specimen of subtotal transversal glossectomy. B. Lobulated and well defined lesion within tong’s wall. C. Epithelioid clear cell proliferation presented alveolar structure. Cells lobules separated by collagen fibrosis bay ( 200). D. Lobulated and well defined tumor with stroma’s fibro-oedmatous change due to neo-adjuvant chemotherapy ( 5). Please cite this article in press as: Baus A, et al. Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report . Ann Chir Plast Esthet (2018), https://doi.org/10.1016/j.anplas.2018.09.001
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A number of biomarkers seem to have been effective in ruling out nodular melanoma [7,25,26]. This main differential diagnosis has a high metastatic risk and requires different treatment modalities than those of CCS [3]. There are few therapeutic guidelines for head and neck soft tissues sarcoma management in adults, surgery remains the gold standard. A monoblock resection with negative margins must be realized. There is no consensus to determine margin width, 2 cm is generally acceptable [27]. In this case, the posterior margin was inferior. Nevertheless, 2 cm posterior margin would have removed the totality of the base of tongue, with such an impossibility to recover an oral alimentation, a high risk of secondary functional laryngectomy. Most authors do not recommend lymph nodes surgery because regional lymph nodes metastases are rare [28]. However, two entities are exceptions: epithelioid sarcoma and clear cell sarcoma that is why we performed a bilateral neck dissection [29]. Bones and lungs are the other common sites of metastatic recurrence [12,30—34]. If resection margins are too close (R0/R1), radiotherapy is indicated since it has shown better local control of the tumor [4,35,36]. Neo-adjuvant chemotherapy for head and neck sarcoma is commonly realized in case of advanced inoperable tumor, or for tumor regression to achieve a R0/R1 resection or to limit a mutilating surgery [37]. However, certain histologic subtypes as soft-tissue alveolar sarcoma, well differentied liposarcoma and clear-cell sarcoma are generally less responsive to chemotherapy [27,38]. The lesion’s size is also of importance as it determines the adjuvant therapeutic strategy for surgery, for instance the initiation of chemotherapy for tumors larger than 5 cm in diameter [39,40]. In our case, the non-response to neoadjuvant chemotherapy lead to propose radiotherapy alone in postoperative care.
Figure 4
All these considerations lead to mutilating surgeries, particularly for head and neck sites, confirming the key role of reconstruction techniques. The main goals of reconstructive surgery following head and neck cancer are multiple. The volumes and the reliefs of the oral cavity have to be maintained. The dead spaces have to be filled. Moreover, functional and volumetric ‘‘ad integrum’’ recovery should always be sought after as well as limiting as much as possible postoperative complications. Tongue reconstructions are of utmost importance as it plays a social (speech, oral hygiene) but also nutritional (chewing, feeding) role. Overall improvement of the survival rate of patients with tongue cancer further reinforces the fact that postoperative quality of life has been to taken into account/ the importance of the concept of postoperative quality of life. Guidelines regarding the type of technique to choose depending on the loss of tongue substance have been proposed and are commonly used for tongue reconstruction and rehabilitation [41]. As such, many technical variants have been described. Free flaps, thanks to their plasticity, their ease of use, the low morbidity of the donor site as well as the possibility of providing well-vascularized composite tissues, play a key role. In 1989, Koshima and Soeda [15] were the first to use a fascio-cutaneous perforating DIEP flap in Head and neck reconstruction. Mostly used in breast reconstruction, this flap has the advantage of bringing a large volume with a low morbidity of the donor site. Based on these arguments, Yano et al. [42,43] therefore believe that the DIEP flap is the flap of choice in pediatric tongue reconstruction. However, compared to the musculo-cutaneous flaps, it should be noted that the cutaneous and adipose tissues are less vascularized which could increase postoperative complications like infections or postradiation oro-cutaneous fistulas [42]. Furthermore, loss of volume can occur over the time and be
A—B. Two-month postoperative flap appearance. C. Ventral surface of the flap. Note the limited lingual elevation.
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Two-month postoperative view of the donor site.
aggravated by radiotherapy [44]. DIEPs detractors believe that for Head and neck localizations, thinner and malleable flaps, such as the radial forearm flap [45,46] or the anterolateral thigh flap [16], with their possibility of re-innervation, should be favored for tongue reconstruction/have a preferential place in the indication of tongue reconstruction in the adult. According to our experience, oral tongue resections/ glossectomy give the worst functional results after total glossectomy. The objective of this reconstruction was thus threefold: to bring a sufficient volume to allow a good function (and to limit post radiation volume loss); to ensure sufficient plasticity and malleability for the conformation of the flap 3. To limit the aesthetic sequelae of the donor site by ensuring a direct closure (Fig. 5).
Conclusion CCSs are aggressive tumors whose head and neck localization is extremely rare. Their treatment remains mainly surgical. The main differential diagnosis that has to be ruled out is melanoma. Early management is primary since a broad excision followed by adjuvant therapies can cause many aesthetic and functional sequelae. Reconstructive surgery is therefore essential but still remains a challenge that requires to carefully choosing the most appropriate solution for the patient, as well as informing him of the expected results. Given the characteristics of patients suffering from CCS (women between 20 and 40 years), and CCS own characteristics (low morbidity of the donor site, adequate volume of the flap and excellent plasticity), the fasciocutaneous free flap ‘‘DIEP’’ taken according to the pattern
of the ‘‘triptych of the cathedral’’ seems to be a viable choice among the range of reconstruction solutions.
Disclosure of interest The authors declare that they have no competing interest.
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Please cite this article in press as: Baus A, et al. Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report . Ann Chir Plast Esthet (2018), https://doi.org/10.1016/j.anplas.2018.09.001