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Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma
G Model ANORL-482; No. of Pages 4
ARTICLE IN PRESS European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2015) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Original article
Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma M. Chirat a , L. Dainese b , S. Fasola c , V. Couloigner a , F. Denoyelle a , E.-N. Garabedian a , N. Leboulanger a,∗ a b c
Service d’ORL et de chirurgie cervico-faciale, hôpital universitaire Necker Enfants Malades, Paris V University, 149, rue de Sèvres, 75015 Paris, France Service d’anatomo-pathologie, hôpital Armand-Trousseau, Paris VI University, 26, avenue du Dr Arnold-Netter, 75012 Paris, France Service d’onco-hématologie, hôpital Armand-Trousseau, Paris VI University, 26, avenue du Dr Arnold-Netter, 75012 Paris, France
a r t i c l e Keywords: Rhabdomyosarcoma Outer ear Auricle Child
i n f o
a b s t r a c t Introduction: Rhabdomyosarcoma (RMS) is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. The main head and neck locations are the base of the skull, nasopharynx, nasal cavity and orbit. An outer ear location is considered extremely rare. We present 3 cases of children, aged 6 to 14 years, presenting with auricular RMS. Case reports: The first child, aged 6, was managed by 4 chemotherapy cycles followed by surgical resection of the tumor bed, completed by 5 further cycles of chemotherapy. The second, aged 14, was managed by 4 chemotherapy cycles followed by external radiation therapy of the tumor bed and lymph node areas, completed by 5 further cycles of chemotherapy. The third, aged 13, was managed by 4 chemotherapy cycles followed by surgery, completed by 5 further cycles of chemotherapy. Discussion: In these 3 patients, the treatment program achieved complete disease control. Prognosis was good, thanks to good surgical access. Diagnosis should be considered in case of unusual progressive swelling in the outer ear. © 2015 Elsevier Masson SAS. All rights reserved.
1. Introduction
2. Material and method
Rhabdomyosarcoma (RMS) is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors [1]. Onset is usually before the age of 12 years [2]. In pediatric populations, 2 main histologic forms are seen: embryonal and alveolar RMS. Pleomorphic RMS is mainly found in adults. In children, 30% to 40% of RMSs develop in the head and neck region, the main locations being the base of the skull, nasopharynx, nasal cavity and orbit [3,4]. RMS is a malignant mesenchymatous tumor growing from primitive cells involved in skeletal muscle differentiation. It may also occur throughout the body, even in organs without striate muscle [1,3]. Outer ear locations are considered extremely rare; to the best of our knowledge, there have been only 3 case reports [4–6]. Rarity, absence of specific symptoms and misleading, sometimes inflammatory and infectious presentation can delay diagnosis and lead to unsuitable primary management. We report 3 new cases managed by a single team, and discuss treatment modalities according to existing international guidelines.
A retrospective analysis was performed on files of patients followed for auricular RMS by the same team in the pediatric ENT departments of the Necker Hospital for Sick Children and Armand-Trousseau Hospital, Paris, France, over a 4-year period from November 2010 to November 2014.
∗ Corresponding author. E-mail address: [email protected] (N. Leboulanger).
3. Results Three children presenting with auricular RMS were treated during the study period. 3.1. Patient #1 A 6-year-old boy presented with swelling of the left concha of 5 months’ evolution. The lesion resembled a cheloid scar, but there was no history of trauma or infection. Given the persistence and growth of the lesion (2 cm long axis at diagnosis) (Fig. 1), biopsy was performed under general anesthesia. Microscopy found skin cover with islands and clusters of malignant tumoral proliferation. The tumor cells were mostly round, with clear cytoplasm and a nucleolated fine chromatin nucleus. There were numerous mitoses. There were also, more rarely,
http://dx.doi.org/10.1016/j.anorl.2015.09.005 1879-7296/© 2015 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Chirat M, et al. Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma. European Annals of Otorhinolaryngology, Head and Neck diseases (2015), http://dx.doi.org/10.1016/j.anorl.2015.09.005
G Model ANORL-482; No. of Pages 4 2
ARTICLE IN PRESS M. Chirat et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2015) xxx–xxx
Extension assessment comprised brain and temporal MRI, head and neck and thoracic contrast-enhanced CT, bone scintigraphy and myelogram. In the left auricle, MRI found an a bumpy contoured oval mass 26 mm high, 13 mm wide and 20 mm thick, showing weak signal on T2 and FLAIR sequences, with strong uptake and relatively well-delineated. Cerebral parenchyma was normal. CT showed the formation of the outer ear soft tissue, without bone extension. There was no cervical adenomegaly or metastatic extension on chest CT or bone scintigraphy. There was thus N−, IRS III localized embryonal RMS of less than 5 cm in a favorable location in a child less than 10 years of age. The multidisciplinary team meeting recommended implementing the EpSSG RMS 2005 standard risk group, subgroup C protocol [7]. The patient therefore initially received 4 cycles of IVA (ifosfamide, vincristine, actinomycin); clinical and radiological response was good after 3 cycles, and surgical resection of the tumor bed was performed. Microscopic inspection of the specimen found no viable residual tumor; all margins were healthy. Resection being macro- and microscopically complete, 5 postoperative cycles of IVA were implemented, without radiation therapy. At the time of writing (36 months’ follow-up), the patient was in complete remission. Secondary auricleplasty may later be considered, depending on the patient’s esthetic desiderata. 3.2. Patient #2 Fig. 1. Patient #1, primary lesion at biopsy.
cells with ribbon- or racket-shaped eosinophil cytoplasm. On immunohistochemistry, about 35% of tumor cells were desmin and myogenin positive (Fig. 2a–c). RT-PCR screening for specific solid tumor translocation was negative. Embryonal RMS was diagnosed.
A 14-year-old boy presented with a lesion of the left concha. It was initially associated with juvenile acne and later attributed to a sebaceous cyst. Several antibiotic and local treatments were without effect; surgical resection was also suggested. The swelling progressively increased over 7 months, and the parents consulted a hospital dermatology department. Examination found a large
Fig. 2. a: patient #1, malignant proliferation comprising islands separated by thin fibrous septa. Cells of various sizes, with poorly visible cytoplasm, round or oval nuclei with jutting nucleoli. Frequent mitoses. Hematoxylin-eosin staining, × 40 magnification; b: patient #1, desmin staining, × 20 magnification: intense membrane and cytoplasm staining and diffuse tumor cell staining; c: patient #1, myogenin staining, × 20 magnification: positive tumor cell nuclei, assessed at 35%.
Please cite this article in press as: Chirat M, et al. Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma. European Annals of Otorhinolaryngology, Head and Neck diseases (2015), http://dx.doi.org/10.1016/j.anorl.2015.09.005
G Model ANORL-482; No. of Pages 4
ARTICLE IN PRESS M. Chirat et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2015) xxx–xxx
3
The multidisciplinary team meeting recommended treatment under EpSSG RMS 2005 standard risk, subgroup C [7]. The patient received 4 cycles of IVA followed by tumor bed resection. Microscopic specimen examination found no residual proliferation, and it was decided to abstain from radiation therapy; treatment was completed by 5 cycles of IVA. At the time of writing (7 months’ follow-up), the patient was in complete remission.
4. Discussion 4.1. Rhabdomyosarcoma
Fig. 3. Patient #2, primary lesion.
budding conchal lesion, 25 mm high and 20 mm wide and also retro-auricular adenopathy (Fig. 3). Biopsy was performed. On microscopy, the skin sample was massively infiltrated through the entire dermis by tumoral proliferation of malignant aspect. Screening for specific translocation was negative. Left auricle embryonal RMS was diagnosed. Extension assessment found deep infiltration of the external ear canal floor and posterior wall. There was no bone or remote extension. MRI also found 3 necrotic adenopathies: pre-tragal (17 × 11 mm), intra-parotid (20 × 10 mm) and spinal (8 × 8 mm). Superior cervical adenopathy aspiration confirmed lymph node invasion. There was thus N+, IRS III localized embryonal RMS of less than 5 cm in a favorable location in a child over 10 years of age. The multidisciplinary team meeting recommended treatment under EpSSG RMS 2005 high risk, subgroup F [7]. The patient received 4 cycles of IVA, with good clinical and radiological response. Surgical resection was proposed but rejected by the patient and parents; treatment was therefore completed by 54 Gy external radiation to the tumor bed, 50 Gy to the cervical lymph nodes and 5 cycles of IVA. At the time of writing (28 months’ follow-up), the patient was in complete remission. 3.3. Patient #3 A 13-year-old girl presented with a painless purplish posterior left auricle lesion of several months’ evolution, attributed to a retro-auricular cyst. Surgical resection was performed. Macroscopic examination of the specimen found a 21 × 20 × 15 mm well-delineated multinodular solid non-cystic tumoral lesion diagnosed by pathologic analysis as embryonal RMS. Translocation analysis was negative. Extension assessment found no pulmonary, bone or medullary extension, but resection was considered microscopically incomplete. There was thus N−, IRS III localized embryonal RMS of less than 5 cm in a favorable location in a child over 10 years of age.
RMS develops from primitive mesenchymatous cells which differentiate into striate muscle. It can be found throughout the body, including in tissue without striate muscle. The main histologic subtypes are embryonal (60%) and alveolar (20%). The former most often involves the nasopharyngeal mucosa, inner ear, urogenital tract and digestive apparatus; the latter most often involves the trunk and limbs [1]. The embryonal subtype is considered low or standard risk, whereas the alveolar subtype is high risk, with poorer prognosis [5]. The 3 present cases were of embryonal RMS. The literature contains only one case of alveolar auricular RMS, in a 12-year-old [6]; she was managed by surgical resection, radiation therapy and chemotherapy associating vincristine, actinomycin and cyclophosphamide, and was in complete remission at 22 months’ follow-up. 4.2. Clinical presentation To the best of our knowledge, only 3 cases of auricular RMS have previously been reported [4–6]; atypical aspect, clinical presentation and unusual location delayed diagnosis by several months. Any lesion of unusual aspect rapidly developing in the auricle should therefore suggest a diagnosis of RMS, even in children, indicating biopsy. 4.3. Extension assessment Initial imaging usually comprises CT and MRI. CT assesses temporal bone cortex destruction. MRI assesses soft-tissue extension, lesion size and limits and lymph node extension, and then treatment response and residue and allows early detection of locoregional recurrence during surveillance [3]. Extension assessment is completed by bone scintigraphy, chest CT and osteomedullary biopsy to screen for second locations. 4.4. Treatment Treatment of non-metastatic RMS follows the EpSSG RMS 2005 protocol [7], with subgroup classification after initial work-up according to histologic type (alveolar or not), initial post-surgical status (complete, macro- or microscopically incomplete resection), location (favorable or not), lymph node invasion, patient age (less or greater than 10 years) and tumor size (less or greater than 5 cm). Four groups of increasing risk are distinguished. The favorable prognosis group comprises favorable anatomopathology (non-alveolar type), complete primary resection, absence of lymph node invasion, favorable age (< 10 years) and tumor < 5 cm. Treatment is completed by 8 cycles of vincristine and actinomycin D spread over 22 weeks. No revision surgery or radiation therapy is indicated. The other 3 groups (standard, high and very high risk) receive similar treatment, with 9 cycles of ifosfamide, vincristine and actinomycin D spread over 25 weeks. Local treatment by surgery
Please cite this article in press as: Chirat M, et al. Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma. European Annals of Otorhinolaryngology, Head and Neck diseases (2015), http://dx.doi.org/10.1016/j.anorl.2015.09.005
G Model ANORL-482; No. of Pages 4
ARTICLE IN PRESS M. Chirat et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2015) xxx–xxx
4
and/or radiation therapy is proposed at the 12th week (after the 4th chemotherapy cycle) in case of incomplete primary resection; radiation doses range from 36 to 50 Gy, according to resection margins, location and treatment response. Exceptions may be made for very young children or especially sensitive locations [7]. Very high-risk cases receive maintenance therapy. The auricle is considered as a favorable site, inasmuch as the lesion is usually accessible to complete resection. The second aim is to achieve complete primary resection with healthy margins to avoid revision surgery, which can be difficult, requiring large-scale facial amputation, leaving blemishes, and radiation therapy, with risk of long-term side-effects.
5. Conclusion Auricular RMS is rare but should be suspected in case of unusual progressive outer ear tumefaction. Biopsy should be performed rapidly in case of the slightest doubt. Prognosis is good, as the site is accessible to surgery. Treatment follows a rigorous international
protocol associating surgery, chemotherapy and sometimes radiation therapy. Disclosure of interest The authors declare that they have no competing interest. References [1] McCarville MB, et al. Rhabdomyosarcoma in pediatric patients: the good, the bad, and the unusual. Am J Roentgenol 2001;176:1563–9. [2] Yousem DM, et al. Rhabdomyosarcomas in the head and neck: MR imaging evaluation. Radiology 1990;177:683–6. [3] Freling NJM, et al. Imaging findings in craniofacial childhood rhabdomyosarcoma. Pediatr Radiol 2010;40:1723–38. [4] Crozier E, et al. Embryonal rhabdomyosarcoma of the auricule in a child. Pediatr Int 2012;54:945–7. [5] Burrows NP, et al. Auricular embryonal rhabdomyosarcoma. Dermatology 1994;189:301–3. [6] Ragsdale BD, et al. Alveolar rhabdomyosarcoma on the external ear: a case report. J Cutan Pathol 2009;36:267–9. [7] Protocole of the Europeean Pediatric Soft Tissue Sarcoma Study Group 2005. http://www.epssgassociation.it/index.php.
Please cite this article in press as: Chirat M, et al. Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma. European Annals of Otorhinolaryngology, Head and Neck diseases (2015), http://dx.doi.org/10.1016/j.anorl.2015.09.005
ARTICLE IN PRESS European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2015) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Original article
Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma M. Chirat a , L. Dainese b , S. Fasola c , V. Couloigner a , F. Denoyelle a , E.-N. Garabedian a , N. Leboulanger a,∗ a b c
Service d’ORL et de chirurgie cervico-faciale, hôpital universitaire Necker Enfants Malades, Paris V University, 149, rue de Sèvres, 75015 Paris, France Service d’anatomo-pathologie, hôpital Armand-Trousseau, Paris VI University, 26, avenue du Dr Arnold-Netter, 75012 Paris, France Service d’onco-hématologie, hôpital Armand-Trousseau, Paris VI University, 26, avenue du Dr Arnold-Netter, 75012 Paris, France
a r t i c l e Keywords: Rhabdomyosarcoma Outer ear Auricle Child
i n f o
a b s t r a c t Introduction: Rhabdomyosarcoma (RMS) is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. The main head and neck locations are the base of the skull, nasopharynx, nasal cavity and orbit. An outer ear location is considered extremely rare. We present 3 cases of children, aged 6 to 14 years, presenting with auricular RMS. Case reports: The first child, aged 6, was managed by 4 chemotherapy cycles followed by surgical resection of the tumor bed, completed by 5 further cycles of chemotherapy. The second, aged 14, was managed by 4 chemotherapy cycles followed by external radiation therapy of the tumor bed and lymph node areas, completed by 5 further cycles of chemotherapy. The third, aged 13, was managed by 4 chemotherapy cycles followed by surgery, completed by 5 further cycles of chemotherapy. Discussion: In these 3 patients, the treatment program achieved complete disease control. Prognosis was good, thanks to good surgical access. Diagnosis should be considered in case of unusual progressive swelling in the outer ear. © 2015 Elsevier Masson SAS. All rights reserved.
1. Introduction
2. Material and method
Rhabdomyosarcoma (RMS) is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors [1]. Onset is usually before the age of 12 years [2]. In pediatric populations, 2 main histologic forms are seen: embryonal and alveolar RMS. Pleomorphic RMS is mainly found in adults. In children, 30% to 40% of RMSs develop in the head and neck region, the main locations being the base of the skull, nasopharynx, nasal cavity and orbit [3,4]. RMS is a malignant mesenchymatous tumor growing from primitive cells involved in skeletal muscle differentiation. It may also occur throughout the body, even in organs without striate muscle [1,3]. Outer ear locations are considered extremely rare; to the best of our knowledge, there have been only 3 case reports [4–6]. Rarity, absence of specific symptoms and misleading, sometimes inflammatory and infectious presentation can delay diagnosis and lead to unsuitable primary management. We report 3 new cases managed by a single team, and discuss treatment modalities according to existing international guidelines.
A retrospective analysis was performed on files of patients followed for auricular RMS by the same team in the pediatric ENT departments of the Necker Hospital for Sick Children and Armand-Trousseau Hospital, Paris, France, over a 4-year period from November 2010 to November 2014.
∗ Corresponding author. E-mail address: [email protected] (N. Leboulanger).
3. Results Three children presenting with auricular RMS were treated during the study period. 3.1. Patient #1 A 6-year-old boy presented with swelling of the left concha of 5 months’ evolution. The lesion resembled a cheloid scar, but there was no history of trauma or infection. Given the persistence and growth of the lesion (2 cm long axis at diagnosis) (Fig. 1), biopsy was performed under general anesthesia. Microscopy found skin cover with islands and clusters of malignant tumoral proliferation. The tumor cells were mostly round, with clear cytoplasm and a nucleolated fine chromatin nucleus. There were numerous mitoses. There were also, more rarely,
http://dx.doi.org/10.1016/j.anorl.2015.09.005 1879-7296/© 2015 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Chirat M, et al. Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma. European Annals of Otorhinolaryngology, Head and Neck diseases (2015), http://dx.doi.org/10.1016/j.anorl.2015.09.005
G Model ANORL-482; No. of Pages 4 2
ARTICLE IN PRESS M. Chirat et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2015) xxx–xxx
Extension assessment comprised brain and temporal MRI, head and neck and thoracic contrast-enhanced CT, bone scintigraphy and myelogram. In the left auricle, MRI found an a bumpy contoured oval mass 26 mm high, 13 mm wide and 20 mm thick, showing weak signal on T2 and FLAIR sequences, with strong uptake and relatively well-delineated. Cerebral parenchyma was normal. CT showed the formation of the outer ear soft tissue, without bone extension. There was no cervical adenomegaly or metastatic extension on chest CT or bone scintigraphy. There was thus N−, IRS III localized embryonal RMS of less than 5 cm in a favorable location in a child less than 10 years of age. The multidisciplinary team meeting recommended implementing the EpSSG RMS 2005 standard risk group, subgroup C protocol [7]. The patient therefore initially received 4 cycles of IVA (ifosfamide, vincristine, actinomycin); clinical and radiological response was good after 3 cycles, and surgical resection of the tumor bed was performed. Microscopic inspection of the specimen found no viable residual tumor; all margins were healthy. Resection being macro- and microscopically complete, 5 postoperative cycles of IVA were implemented, without radiation therapy. At the time of writing (36 months’ follow-up), the patient was in complete remission. Secondary auricleplasty may later be considered, depending on the patient’s esthetic desiderata. 3.2. Patient #2 Fig. 1. Patient #1, primary lesion at biopsy.
cells with ribbon- or racket-shaped eosinophil cytoplasm. On immunohistochemistry, about 35% of tumor cells were desmin and myogenin positive (Fig. 2a–c). RT-PCR screening for specific solid tumor translocation was negative. Embryonal RMS was diagnosed.
A 14-year-old boy presented with a lesion of the left concha. It was initially associated with juvenile acne and later attributed to a sebaceous cyst. Several antibiotic and local treatments were without effect; surgical resection was also suggested. The swelling progressively increased over 7 months, and the parents consulted a hospital dermatology department. Examination found a large
Fig. 2. a: patient #1, malignant proliferation comprising islands separated by thin fibrous septa. Cells of various sizes, with poorly visible cytoplasm, round or oval nuclei with jutting nucleoli. Frequent mitoses. Hematoxylin-eosin staining, × 40 magnification; b: patient #1, desmin staining, × 20 magnification: intense membrane and cytoplasm staining and diffuse tumor cell staining; c: patient #1, myogenin staining, × 20 magnification: positive tumor cell nuclei, assessed at 35%.
Please cite this article in press as: Chirat M, et al. Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma. European Annals of Otorhinolaryngology, Head and Neck diseases (2015), http://dx.doi.org/10.1016/j.anorl.2015.09.005
G Model ANORL-482; No. of Pages 4
ARTICLE IN PRESS M. Chirat et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2015) xxx–xxx
3
The multidisciplinary team meeting recommended treatment under EpSSG RMS 2005 standard risk, subgroup C [7]. The patient received 4 cycles of IVA followed by tumor bed resection. Microscopic specimen examination found no residual proliferation, and it was decided to abstain from radiation therapy; treatment was completed by 5 cycles of IVA. At the time of writing (7 months’ follow-up), the patient was in complete remission.
4. Discussion 4.1. Rhabdomyosarcoma
Fig. 3. Patient #2, primary lesion.
budding conchal lesion, 25 mm high and 20 mm wide and also retro-auricular adenopathy (Fig. 3). Biopsy was performed. On microscopy, the skin sample was massively infiltrated through the entire dermis by tumoral proliferation of malignant aspect. Screening for specific translocation was negative. Left auricle embryonal RMS was diagnosed. Extension assessment found deep infiltration of the external ear canal floor and posterior wall. There was no bone or remote extension. MRI also found 3 necrotic adenopathies: pre-tragal (17 × 11 mm), intra-parotid (20 × 10 mm) and spinal (8 × 8 mm). Superior cervical adenopathy aspiration confirmed lymph node invasion. There was thus N+, IRS III localized embryonal RMS of less than 5 cm in a favorable location in a child over 10 years of age. The multidisciplinary team meeting recommended treatment under EpSSG RMS 2005 high risk, subgroup F [7]. The patient received 4 cycles of IVA, with good clinical and radiological response. Surgical resection was proposed but rejected by the patient and parents; treatment was therefore completed by 54 Gy external radiation to the tumor bed, 50 Gy to the cervical lymph nodes and 5 cycles of IVA. At the time of writing (28 months’ follow-up), the patient was in complete remission. 3.3. Patient #3 A 13-year-old girl presented with a painless purplish posterior left auricle lesion of several months’ evolution, attributed to a retro-auricular cyst. Surgical resection was performed. Macroscopic examination of the specimen found a 21 × 20 × 15 mm well-delineated multinodular solid non-cystic tumoral lesion diagnosed by pathologic analysis as embryonal RMS. Translocation analysis was negative. Extension assessment found no pulmonary, bone or medullary extension, but resection was considered microscopically incomplete. There was thus N−, IRS III localized embryonal RMS of less than 5 cm in a favorable location in a child over 10 years of age.
RMS develops from primitive mesenchymatous cells which differentiate into striate muscle. It can be found throughout the body, including in tissue without striate muscle. The main histologic subtypes are embryonal (60%) and alveolar (20%). The former most often involves the nasopharyngeal mucosa, inner ear, urogenital tract and digestive apparatus; the latter most often involves the trunk and limbs [1]. The embryonal subtype is considered low or standard risk, whereas the alveolar subtype is high risk, with poorer prognosis [5]. The 3 present cases were of embryonal RMS. The literature contains only one case of alveolar auricular RMS, in a 12-year-old [6]; she was managed by surgical resection, radiation therapy and chemotherapy associating vincristine, actinomycin and cyclophosphamide, and was in complete remission at 22 months’ follow-up. 4.2. Clinical presentation To the best of our knowledge, only 3 cases of auricular RMS have previously been reported [4–6]; atypical aspect, clinical presentation and unusual location delayed diagnosis by several months. Any lesion of unusual aspect rapidly developing in the auricle should therefore suggest a diagnosis of RMS, even in children, indicating biopsy. 4.3. Extension assessment Initial imaging usually comprises CT and MRI. CT assesses temporal bone cortex destruction. MRI assesses soft-tissue extension, lesion size and limits and lymph node extension, and then treatment response and residue and allows early detection of locoregional recurrence during surveillance [3]. Extension assessment is completed by bone scintigraphy, chest CT and osteomedullary biopsy to screen for second locations. 4.4. Treatment Treatment of non-metastatic RMS follows the EpSSG RMS 2005 protocol [7], with subgroup classification after initial work-up according to histologic type (alveolar or not), initial post-surgical status (complete, macro- or microscopically incomplete resection), location (favorable or not), lymph node invasion, patient age (less or greater than 10 years) and tumor size (less or greater than 5 cm). Four groups of increasing risk are distinguished. The favorable prognosis group comprises favorable anatomopathology (non-alveolar type), complete primary resection, absence of lymph node invasion, favorable age (< 10 years) and tumor < 5 cm. Treatment is completed by 8 cycles of vincristine and actinomycin D spread over 22 weeks. No revision surgery or radiation therapy is indicated. The other 3 groups (standard, high and very high risk) receive similar treatment, with 9 cycles of ifosfamide, vincristine and actinomycin D spread over 25 weeks. Local treatment by surgery
Please cite this article in press as: Chirat M, et al. Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma. European Annals of Otorhinolaryngology, Head and Neck diseases (2015), http://dx.doi.org/10.1016/j.anorl.2015.09.005
G Model ANORL-482; No. of Pages 4
ARTICLE IN PRESS M. Chirat et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2015) xxx–xxx
4
and/or radiation therapy is proposed at the 12th week (after the 4th chemotherapy cycle) in case of incomplete primary resection; radiation doses range from 36 to 50 Gy, according to resection margins, location and treatment response. Exceptions may be made for very young children or especially sensitive locations [7]. Very high-risk cases receive maintenance therapy. The auricle is considered as a favorable site, inasmuch as the lesion is usually accessible to complete resection. The second aim is to achieve complete primary resection with healthy margins to avoid revision surgery, which can be difficult, requiring large-scale facial amputation, leaving blemishes, and radiation therapy, with risk of long-term side-effects.
5. Conclusion Auricular RMS is rare but should be suspected in case of unusual progressive outer ear tumefaction. Biopsy should be performed rapidly in case of the slightest doubt. Prognosis is good, as the site is accessible to surgery. Treatment follows a rigorous international
protocol associating surgery, chemotherapy and sometimes radiation therapy. Disclosure of interest The authors declare that they have no competing interest. References [1] McCarville MB, et al. Rhabdomyosarcoma in pediatric patients: the good, the bad, and the unusual. Am J Roentgenol 2001;176:1563–9. [2] Yousem DM, et al. Rhabdomyosarcomas in the head and neck: MR imaging evaluation. Radiology 1990;177:683–6. [3] Freling NJM, et al. Imaging findings in craniofacial childhood rhabdomyosarcoma. Pediatr Radiol 2010;40:1723–38. [4] Crozier E, et al. Embryonal rhabdomyosarcoma of the auricule in a child. Pediatr Int 2012;54:945–7. [5] Burrows NP, et al. Auricular embryonal rhabdomyosarcoma. Dermatology 1994;189:301–3. [6] Ragsdale BD, et al. Alveolar rhabdomyosarcoma on the external ear: a case report. J Cutan Pathol 2009;36:267–9. [7] Protocole of the Europeean Pediatric Soft Tissue Sarcoma Study Group 2005. http://www.epssgassociation.it/index.php.
Please cite this article in press as: Chirat M, et al. Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma. European Annals of Otorhinolaryngology, Head and Neck diseases (2015), http://dx.doi.org/10.1016/j.anorl.2015.09.005