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A facial inflammatory myofibroblastic tumour in a 6-year-old girl: Plastic surgery lessons from a rare case
Journal of Cranio-Maxillo-Facial Surgery 39 (2011) 141e144
Contents lists available at ScienceDirect
Journal of Cranio-Maxillo-Facial Surgery journal homepage: www.jcmfs.com
A facial inflammatory myofibroblastic tumour in a 6-year-old girl: Plastic surgery lessons from a rare case Ali Modarressi a, *, Giorgio Pietramaggiori a, Thierry Bezzola a, Sylvain Lacroix b, Michel Richter c, Benedict Rilliet d, Brigitte Pittet a a
Plastic, Reconstructive and Aesthetic Surgery Unit, Geneva University Hospitals and Faculty of Medicine, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14, Switzerland ENT Unit, Geneva University Hospitals and Faculty of Medicine, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14, Switzerland Maxillofacial Surgery Unit, Geneva University Hospitals and Faculty of Medicine, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14, Switzerland d Neurosurgery Unit, Geneva University Hospitals and Faculty of Medicine, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14, Switzerland b c
a r t i c l e i n f o
a b s t r a c t
Article history: Paper received 1 June 2010 Accepted 26 November 2010
Inflammatory myofibroblastic tumours (IMT) are rare, challenging lesions with respect to differential diagnosis, biological behaviour and treatment. We reviewed the literature and report a unique case of a large (9 8 8 cm) IMT in the nasal region of a 6-year-old girl responsible for important facial deformation. Following surgical resection, without any craniofacial reconstruction, the dysmorphism regressed spontaneously with a good result at 2 years of follow-up. To date, this is the largest IMT reported. Surprisingly, after simple excision of the tumour the craniofacial bones and soft tissues regained spontaneously their normal anatomical position only after a few months. The bone plasticity was likely due to the young age of patient and the preservation of periosteum and muscles during the surgical excision. We conclude that a conservative approach may be considered as alternative to reconstructive surgery in particular cases in young subjects. Ó 2010 European Association for Cranio-Maxillo-Facial Surgery.
Keywords: Myofibroblastic tumour Bone remodelling Craniofacial plastic surgery
1. Introduction The inflammatory myofibroblastic tumour (IMT) has been defined in the classification of soft tissue tumours as a distinct lesion composed of myofibroblasts and inflammatory infiltrate. This lesion has been primarily described in soft tissues and viscera of children and young adults (Morotti et al., 2005), although no clear age preference has been characterized, with equal incidence in male and female patients (Ribeiro et al., 2001). IMT rarely affects the head and neck region; to date fewer than 30 cases have been reported, mostly in the upper aerodigestive tract. Despite an apparently benign morphological nature, some cases have been reported to have a locally aggressive growth, a tendency to recur (25%) and rarely to metastasise (5%). Complete surgical excision of the tumour has proved to be the only effective treatment, while chemotherapy or radiotherapy has been shown to be ineffective. The optimal duration of follow-up has not been defined (Morotti et al., 2005), with recurrences occurring at variable time-points following primary surgery.
* Corresponding author. Tel.: þ41 22 372 79 97; fax: þ41 372 80 05. E-mail address: [email protected] (A. Modarressi).
Here, we report a rare case of a large IMT of the nasal cavity causing a significant deformation of the facial structures, which resolved spontaneously after simple excision of the tumour. 2. Case report A 6-year-old girl from Togo was referred by a humanitarian association for a rapidly growing mass in the central facial region for 2 years. A partial resection was performed in Togo, histopathology revealing a juvenile myofibroma. This first operation was followed by a massive growth of the lesion in the central facial region inducing an important and rapid deformation of the face over a period of 6 months. At arrival in Switzerland, physical examination revealed a mass elongating the centro-facial area with total obstruction of nostrils, telecanthus (6,5 cm) and exophtalmos, without lymph node involvement (Fig. 1). CT scan and MRI showed a large heterogeneous mass (9 8 8 cm) centred in the nasal cavity and occupying the entire central facial region (Fig. 2). Wedge biopsy revealed an IMT. To reduce bleeding risk during the surgical procedure, a selective, pre-operative embolisation of both internal maxillary arteries was performed. To avoid a facial scar, we started the operation by a bicoronal and intraoral approach. Considering the size of the tumour and its
1010-5182/$ e see front matter Ó 2010 European Association for Cranio-Maxillo-Facial Surgery. doi:10.1016/j.jcms.2010.11.015
142
A. Modarressi et al. / Journal of Cranio-Maxillo-Facial Surgery 39 (2011) 141e144
Fig. 1. Left: In 2005, before the apparition of the tumour. Middle: In may 2007, before the operation: nasal deformation with tumour extrusion through nostrils, hypertelorism and exophtalmos. Right: In 2009: 2 years post-operative in Africa: no sign of recurrence.
Fig. 2. Left: MRI: heterogeneous mass (9 8 8 cm) centred in the nasal cavity, occupying the entire centro-facial region with orbital spacing, displacement of internal rectus muscles and optical nerves occurred at the ocular level. It was associated with an important deformation of the facial skeleton, which was pushed aside by the growing mass. We observed complete loss of nasal cavity, cribriform and papyraceal plates and remodelling of sphenoidal and palatine bones. Right: MRI 9 months post-operation.
localization, we had to perform para-nasal and trans-facial incisions to resect it in toto with preservation of the periosteum and muscles. It presented as a white fragile tissue weighting 250 g. While waiting for definitive histopathological analysis, considering the possibility of early recurrence, and to give the opportunity to the bone plasticity to reconstruct the defect, the decision was taken not to perform any immediate reconstructive surgery. Histological examination confirmed the diagnosis of IMT. After surgery, we noted a rapid regression of the deformation and gradual amelioration of the morphological appearance, with progressive normalization of the middle face. The anatomical structures became spontaneously normal with symmetrical positioning over the first month of follow-up. Radiological control at 9 months after excision, confirmed the progressive re-appearance of the normal craniofacial anatomical bony structures (Figs. 2 and 3).
However, a local tumour growth, due to incomplete primary resection, was found in the naso-ethmoidal cavity which was resected by endoscopy. The latest CT scan at 2 years of follow-up did not show the presence of any recurrence, with re-ossification of the bony defect and normalization of the craniofacial skeleton (Fig. 2). 3. Discussion IMTs are histologically characterized by dominant myofibroblasts invasion and variable inflammatory infiltrate. They are generally benign, but sometimes can be locally aggressive. Myofibroblasts, differentiated fibroblasts expressing a-smooth muscle actin (Hinz et al., 2007a), contribute to physiological wound healing. Their absence has been associated
A. Modarressi et al. / Journal of Cranio-Maxillo-Facial Surgery 39 (2011) 141e144
143
Fig. 3. 3D reconstruction of the skull bones. Left: Before the operation, important remodelling of the middle facial skeleton that is pushed aside by the growing mass with complete destruction of nasal cavity, cribriform and papyraceal plates; and deformation of sphenoidal and palatine bones. Right: 9 months after the tumour excision: spontaneous regrowth of the bones of the nasal cavity.
with development of chronic wounds, and their over activation causes the vast majority of fibrocontractive diseases (Hinz et al., 2007a, Hinz, 2007b). Interestingly, these events are rarely associated with tumour growth and only IMTs are characterized histologically by uncontrolled tumoural proliferation of myofibroblasts. This is the largest IMT in the nasal cavity in literature. The tumour significantly displaced and distorted centro-facial bones and soft tissues during a short period of aggressive growth. However, after surgical excision without any reconstructive surgery, craniofacial bones and soft tissues returned to their normal anatomic structure after only few months. The deformation noted in this patient has to be related to centripetal pressure secondary to the expansion of the lesion and not to the local invasion of the tumour. Once this was enucleated, all the surrounding tissues gradually regained the normal anatomical position, revealing a surprisingly high elasticity and impressive memory of the bones. Bones, when subjected to stress can be deformed. The length and intensity of the mechanical stress determine whether the bone would regain its original shape by elastic contraction or whether it will respond as plastic material. In this case, the gradual growth of the tumour mass with constant low intensity mechanical forces in the central facial area induced an elastic deformation. The decision to treat this lesion only by tumour resection turned out to be appropriate, since a nearly reconstructive approach would have been invasive with less harmonic aesthetic and functional results. These results should be taken into consideration when treating non-invasive lesions in young patients. It is established that skull bones in young children, being associated with growth and large shape changes, are highly compliant, more than adult bones. After extensive excisions, the preservation of periosteum and muscles is essential to retrieve the physiological aspect (Cestero et al., 1976; Gedrange and Harzer, 2004; Pepicelli et al., 2005; Babuccu et al., 2009).
Finally, in this case we aimed to be as minimally invasive as possible as suggested by Lund et al. (Lund et al., 2010), but we had to use a trans-facial incision to perform in toto resection of this benign tumour which however presents a high rate of recurrence in case of remaining tumour tissue. In spite of this incision in a young black girl, the aesthetic result is very good at 2 years follow-up. 4. Conclusion In plastic surgery, several pathologies can be compared with this case for the tendency to deform craniofacial areas in young patients, such as the Recklinghausen disease, vascular malformations, encephaloceles and the craniosynosis. From this study, we can argue that in many of these cases it may be possible to reduce the aggressiveness of the reconstructive surgery or to defer it in time, as the regenerative abilities of skull bones of young children are surprisingly high. In absence of any recurrence, we can confirm that gold standard treatment of IMT, as it has been described in the literature, is a complete surgical excision as the only treatment, even in the cases of large tumours. However, a longer follow-up is warranted to evaluate the tendency of tumour recurrence. Funding None. Conflict of interest None. References Babuccu B, Babuccu O, Yurdakan G, Ankarali H: The effect of the Botulinum toxin-A on craniofacial development: an experimental study. Ann Plast Surg 63: 449e456, 2009 Cestero Jr HJ, Salyer KE, Johns DF: The periosteum and craniofacial growth. Surg Forum 27: 556e558, 1976
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A. Modarressi et al. / Journal of Cranio-Maxillo-Facial Surgery 39 (2011) 141e144
Gedrange T, Harzer W: Muscle influence on postnatal craniofacial development and diagnostics. J Orofac Orthop 65: 451e466, 2004 Hinz B, Phan SH, Thannickal VJ, Galli A, Bochaton-Piallat ML, Gabbiani G: The myofibroblast: one function, multiple origins. Am J Pathol 170: 1807e1816, 2007a Hinz B: Formation and function of the myofibroblast during tissue repair. J Invest Dermatol 127: 526e537, 2007b Lund VJ, Stammberger H, Nicolai P, Castelnuovo P, Beal T, Beham A, et al: European position paper on endoscopic management of tumours of the nose, paranasal sinuses and skull base. Rhinol Suppl 22(1): 1e143, 2010
Morotti RA, Legman MD, Kerkar N, Pawel BR, Sanger WG, Coffin CM: Pediatric inflammatory myofibroblastic tumour with late metastasis to the lung: case report and review of the literature. Pediatr Dev Pathol 8: 224e229, 2005 Pepicelli A, Woods M, Briggs C: The mandibular muscles and their importance in orthodontics: a contemporary review. Am J Orthod Dentofacial Orthop 128: 774e780, 2005 Ribeiro AC, Joshi VM, Funkhouser WK, Mukherji SK: Inflammatory myofibroblastic tumour involving the pterygopalatine fossa. AJNR Am J Neuroradiol 22: 518e520, 2001
Contents lists available at ScienceDirect
Journal of Cranio-Maxillo-Facial Surgery journal homepage: www.jcmfs.com
A facial inflammatory myofibroblastic tumour in a 6-year-old girl: Plastic surgery lessons from a rare case Ali Modarressi a, *, Giorgio Pietramaggiori a, Thierry Bezzola a, Sylvain Lacroix b, Michel Richter c, Benedict Rilliet d, Brigitte Pittet a a
Plastic, Reconstructive and Aesthetic Surgery Unit, Geneva University Hospitals and Faculty of Medicine, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14, Switzerland ENT Unit, Geneva University Hospitals and Faculty of Medicine, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14, Switzerland Maxillofacial Surgery Unit, Geneva University Hospitals and Faculty of Medicine, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14, Switzerland d Neurosurgery Unit, Geneva University Hospitals and Faculty of Medicine, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14, Switzerland b c
a r t i c l e i n f o
a b s t r a c t
Article history: Paper received 1 June 2010 Accepted 26 November 2010
Inflammatory myofibroblastic tumours (IMT) are rare, challenging lesions with respect to differential diagnosis, biological behaviour and treatment. We reviewed the literature and report a unique case of a large (9 8 8 cm) IMT in the nasal region of a 6-year-old girl responsible for important facial deformation. Following surgical resection, without any craniofacial reconstruction, the dysmorphism regressed spontaneously with a good result at 2 years of follow-up. To date, this is the largest IMT reported. Surprisingly, after simple excision of the tumour the craniofacial bones and soft tissues regained spontaneously their normal anatomical position only after a few months. The bone plasticity was likely due to the young age of patient and the preservation of periosteum and muscles during the surgical excision. We conclude that a conservative approach may be considered as alternative to reconstructive surgery in particular cases in young subjects. Ó 2010 European Association for Cranio-Maxillo-Facial Surgery.
Keywords: Myofibroblastic tumour Bone remodelling Craniofacial plastic surgery
1. Introduction The inflammatory myofibroblastic tumour (IMT) has been defined in the classification of soft tissue tumours as a distinct lesion composed of myofibroblasts and inflammatory infiltrate. This lesion has been primarily described in soft tissues and viscera of children and young adults (Morotti et al., 2005), although no clear age preference has been characterized, with equal incidence in male and female patients (Ribeiro et al., 2001). IMT rarely affects the head and neck region; to date fewer than 30 cases have been reported, mostly in the upper aerodigestive tract. Despite an apparently benign morphological nature, some cases have been reported to have a locally aggressive growth, a tendency to recur (25%) and rarely to metastasise (5%). Complete surgical excision of the tumour has proved to be the only effective treatment, while chemotherapy or radiotherapy has been shown to be ineffective. The optimal duration of follow-up has not been defined (Morotti et al., 2005), with recurrences occurring at variable time-points following primary surgery.
* Corresponding author. Tel.: þ41 22 372 79 97; fax: þ41 372 80 05. E-mail address: [email protected] (A. Modarressi).
Here, we report a rare case of a large IMT of the nasal cavity causing a significant deformation of the facial structures, which resolved spontaneously after simple excision of the tumour. 2. Case report A 6-year-old girl from Togo was referred by a humanitarian association for a rapidly growing mass in the central facial region for 2 years. A partial resection was performed in Togo, histopathology revealing a juvenile myofibroma. This first operation was followed by a massive growth of the lesion in the central facial region inducing an important and rapid deformation of the face over a period of 6 months. At arrival in Switzerland, physical examination revealed a mass elongating the centro-facial area with total obstruction of nostrils, telecanthus (6,5 cm) and exophtalmos, without lymph node involvement (Fig. 1). CT scan and MRI showed a large heterogeneous mass (9 8 8 cm) centred in the nasal cavity and occupying the entire central facial region (Fig. 2). Wedge biopsy revealed an IMT. To reduce bleeding risk during the surgical procedure, a selective, pre-operative embolisation of both internal maxillary arteries was performed. To avoid a facial scar, we started the operation by a bicoronal and intraoral approach. Considering the size of the tumour and its
1010-5182/$ e see front matter Ó 2010 European Association for Cranio-Maxillo-Facial Surgery. doi:10.1016/j.jcms.2010.11.015
142
A. Modarressi et al. / Journal of Cranio-Maxillo-Facial Surgery 39 (2011) 141e144
Fig. 1. Left: In 2005, before the apparition of the tumour. Middle: In may 2007, before the operation: nasal deformation with tumour extrusion through nostrils, hypertelorism and exophtalmos. Right: In 2009: 2 years post-operative in Africa: no sign of recurrence.
Fig. 2. Left: MRI: heterogeneous mass (9 8 8 cm) centred in the nasal cavity, occupying the entire centro-facial region with orbital spacing, displacement of internal rectus muscles and optical nerves occurred at the ocular level. It was associated with an important deformation of the facial skeleton, which was pushed aside by the growing mass. We observed complete loss of nasal cavity, cribriform and papyraceal plates and remodelling of sphenoidal and palatine bones. Right: MRI 9 months post-operation.
localization, we had to perform para-nasal and trans-facial incisions to resect it in toto with preservation of the periosteum and muscles. It presented as a white fragile tissue weighting 250 g. While waiting for definitive histopathological analysis, considering the possibility of early recurrence, and to give the opportunity to the bone plasticity to reconstruct the defect, the decision was taken not to perform any immediate reconstructive surgery. Histological examination confirmed the diagnosis of IMT. After surgery, we noted a rapid regression of the deformation and gradual amelioration of the morphological appearance, with progressive normalization of the middle face. The anatomical structures became spontaneously normal with symmetrical positioning over the first month of follow-up. Radiological control at 9 months after excision, confirmed the progressive re-appearance of the normal craniofacial anatomical bony structures (Figs. 2 and 3).
However, a local tumour growth, due to incomplete primary resection, was found in the naso-ethmoidal cavity which was resected by endoscopy. The latest CT scan at 2 years of follow-up did not show the presence of any recurrence, with re-ossification of the bony defect and normalization of the craniofacial skeleton (Fig. 2). 3. Discussion IMTs are histologically characterized by dominant myofibroblasts invasion and variable inflammatory infiltrate. They are generally benign, but sometimes can be locally aggressive. Myofibroblasts, differentiated fibroblasts expressing a-smooth muscle actin (Hinz et al., 2007a), contribute to physiological wound healing. Their absence has been associated
A. Modarressi et al. / Journal of Cranio-Maxillo-Facial Surgery 39 (2011) 141e144
143
Fig. 3. 3D reconstruction of the skull bones. Left: Before the operation, important remodelling of the middle facial skeleton that is pushed aside by the growing mass with complete destruction of nasal cavity, cribriform and papyraceal plates; and deformation of sphenoidal and palatine bones. Right: 9 months after the tumour excision: spontaneous regrowth of the bones of the nasal cavity.
with development of chronic wounds, and their over activation causes the vast majority of fibrocontractive diseases (Hinz et al., 2007a, Hinz, 2007b). Interestingly, these events are rarely associated with tumour growth and only IMTs are characterized histologically by uncontrolled tumoural proliferation of myofibroblasts. This is the largest IMT in the nasal cavity in literature. The tumour significantly displaced and distorted centro-facial bones and soft tissues during a short period of aggressive growth. However, after surgical excision without any reconstructive surgery, craniofacial bones and soft tissues returned to their normal anatomic structure after only few months. The deformation noted in this patient has to be related to centripetal pressure secondary to the expansion of the lesion and not to the local invasion of the tumour. Once this was enucleated, all the surrounding tissues gradually regained the normal anatomical position, revealing a surprisingly high elasticity and impressive memory of the bones. Bones, when subjected to stress can be deformed. The length and intensity of the mechanical stress determine whether the bone would regain its original shape by elastic contraction or whether it will respond as plastic material. In this case, the gradual growth of the tumour mass with constant low intensity mechanical forces in the central facial area induced an elastic deformation. The decision to treat this lesion only by tumour resection turned out to be appropriate, since a nearly reconstructive approach would have been invasive with less harmonic aesthetic and functional results. These results should be taken into consideration when treating non-invasive lesions in young patients. It is established that skull bones in young children, being associated with growth and large shape changes, are highly compliant, more than adult bones. After extensive excisions, the preservation of periosteum and muscles is essential to retrieve the physiological aspect (Cestero et al., 1976; Gedrange and Harzer, 2004; Pepicelli et al., 2005; Babuccu et al., 2009).
Finally, in this case we aimed to be as minimally invasive as possible as suggested by Lund et al. (Lund et al., 2010), but we had to use a trans-facial incision to perform in toto resection of this benign tumour which however presents a high rate of recurrence in case of remaining tumour tissue. In spite of this incision in a young black girl, the aesthetic result is very good at 2 years follow-up. 4. Conclusion In plastic surgery, several pathologies can be compared with this case for the tendency to deform craniofacial areas in young patients, such as the Recklinghausen disease, vascular malformations, encephaloceles and the craniosynosis. From this study, we can argue that in many of these cases it may be possible to reduce the aggressiveness of the reconstructive surgery or to defer it in time, as the regenerative abilities of skull bones of young children are surprisingly high. In absence of any recurrence, we can confirm that gold standard treatment of IMT, as it has been described in the literature, is a complete surgical excision as the only treatment, even in the cases of large tumours. However, a longer follow-up is warranted to evaluate the tendency of tumour recurrence. Funding None. Conflict of interest None. References Babuccu B, Babuccu O, Yurdakan G, Ankarali H: The effect of the Botulinum toxin-A on craniofacial development: an experimental study. Ann Plast Surg 63: 449e456, 2009 Cestero Jr HJ, Salyer KE, Johns DF: The periosteum and craniofacial growth. Surg Forum 27: 556e558, 1976
144
A. Modarressi et al. / Journal of Cranio-Maxillo-Facial Surgery 39 (2011) 141e144
Gedrange T, Harzer W: Muscle influence on postnatal craniofacial development and diagnostics. J Orofac Orthop 65: 451e466, 2004 Hinz B, Phan SH, Thannickal VJ, Galli A, Bochaton-Piallat ML, Gabbiani G: The myofibroblast: one function, multiple origins. Am J Pathol 170: 1807e1816, 2007a Hinz B: Formation and function of the myofibroblast during tissue repair. J Invest Dermatol 127: 526e537, 2007b Lund VJ, Stammberger H, Nicolai P, Castelnuovo P, Beal T, Beham A, et al: European position paper on endoscopic management of tumours of the nose, paranasal sinuses and skull base. Rhinol Suppl 22(1): 1e143, 2010
Morotti RA, Legman MD, Kerkar N, Pawel BR, Sanger WG, Coffin CM: Pediatric inflammatory myofibroblastic tumour with late metastasis to the lung: case report and review of the literature. Pediatr Dev Pathol 8: 224e229, 2005 Pepicelli A, Woods M, Briggs C: The mandibular muscles and their importance in orthodontics: a contemporary review. Am J Orthod Dentofacial Orthop 128: 774e780, 2005 Ribeiro AC, Joshi VM, Funkhouser WK, Mukherji SK: Inflammatory myofibroblastic tumour involving the pterygopalatine fossa. AJNR Am J Neuroradiol 22: 518e520, 2001