Case report 227
Acute Respiratory Distress Due to Malignant Schwannoma. A Case Report M. MeUkoglu J, S. KaravelL'2, T (;olak J, T Gelen2 Departments of I Pediatric Surgery and 2pathology, Medical Faculty, UniversiLy of Akdeniz, AnLalya, Turkey
A 13-year-old boy with acute re piratory distress due to a malignant schwannoma located in the neck, is reported. This case suggested that maJignant schwannoma in the neck can lead to airway obstruction.
Key words
1alignant schwannoma - Malignant primar}' nen'e sheath tumors - Acute respiratory distress
Resume
Les auteurs rapportent l'observation d'un gan;on de 13 ans atteint d'une detresse respiratoire aigue par schwann6me malin localise au niveau du cou. Ce cas suggere que les schwann6mes malins dans le cou peuvent conduire a une obstruction des voies respiratoires.
Mols-eIes
Schwann6me malin - Tumeur maligne primitive de couverture nerveuse - Detresse respiratoire aigue
Zusammenfassung
Ein 13jähriger Junge mit einem malignen Schwannoma im Halsbereich, das zu akuten Atemstörungen führte, wird vorgestellt.
l- - - Schlüsselwörter
Malignes Schwannom - Akute Atemnot
Extracranial neurogenie tumors of the head and neck are relatively rare tumors that are often deceptive and may be mistaken for other disease processes (3, 4, 6, 7, 9, 12, 14, 16, 17). Malignant nerve sheath tumors arise from Schwann cells of neuroectodermal origin, and from fibrocytes of mesodermal origin (3,4,5,7,8,10,11,15). Approximately 6-16 % of malignant schwannomas occur in the head and neck (6, 14, 17). ~10st often they arise from cranial nen'es, upper branches of the brachial plexus, cenrlcal nen'e rools and sympathetic chain. Lymph node metastases are rare but local recurrence and hematogenous spread to the lungs are not uncommon. Treatment of malignant schwannoma involves radicallocal excision as the primary mode of treatment (3, 14, 16, 17). Overa1l5-year survivals have varied from 22-30 % (6).
Case report A 13-year-old bay (B. B.) was admitted to the hospital on September 16, 1988, with acute respiratory distress. He had a six months' history of a rapidly enlarging mass on the mid-front of the neck and increasing respiratory distress during the last fifteen days. On physical examination: he was an ill-looking and conscious boy with respiratory distress. He had minimal cyanosis and mild respiratory distress with supraclavicuJar, substernal and intercostal retractions. There was a 10 x 10 x 5 cm fixed mass on the mid front of neck \vith rubber consistency. The overlying skin of the mass was normal. There was no cervieal, axillary and supraclavicuJar lymphadenopathy. The respiration sounds were found to be decreased at the lower lobes. The heart sounds were normal. In direct laryngoscopy, the voeal cords were normal. The rest of the physieal examination findings was normal. Since he had severe respiratory distress, an emergency operation was decided. At the operation, a necrotic and ruhbery mass of 10 x 8 x 5 cm diameter over the trachea, displacing the lobes of the thyroid, was observed. The mass was resected totally, and a tracheotomy was performed due to the risk of tracheomalacia due Lo compression of the trachea by the tumor mass. The postoperative period was uneventfuJ.
Pathologie findings Recei\ed \ larch 11, 1991 Eur J Pediatr Surg 2 (J 992) 227-229 © Hippokrates \'erlag Stuttgart ,\Iasson Editeur Paris
Gross specimens from the original operation included fragments of friable pink-white tissue, firm to soft, depending on the ab ence or presence of necrosis and haemorrhage. .'vlicroscopical examination of the original specimen showed that the tumor was not truly en-
Downloaded by: Universite de Sherbrooke. Copyrighted material.
Introduction
Summary
Eur J Pediatr urg 2 (1992)
,\1. Meltkoglu et al
eapsulated and in\"aded the th}Toid tissue and surrounding muscle tissue and vessels. The pathologie findings of the ease are typieal of the malignant nerve sheath tumors (Figs. 1,2,3).
Clinical course On the 13th postoperative day the patient was sent horne in excellent eondition. SubsequenUy, the patient underwent two courses of Adriablastine, Endoxan and \'ineristine ehemotherapy. He was followed up at monthly inten·al \\ith computerized tomography and found to be normal. _ 0 reeurrent tumor growth and metastases could be identified. He has been aS}Tnptomatie for a one year follow-up period.
Discussion
The patient reported herein represents a case of malignant sch\\-annoma in the neck. Acute respiratol}' distress due to malignant sch\\"annoma in the neck has been reported before (6, 9, l-l, 16). Our ca e is under the age of 20 years and there is no association \\-ith von Recklinghausen's neurofibromatosis.
Fig. 1 The mallgnant x1400
~chwa~nOfT1a
show ng abnormal mltoses (>-I E
[n our case, the tumor mass appeared to be encapsulated, but lhis pseudo-encapsulation \\'as secondal}' to compression of surrounding structures and infiltration of epineurium ,,-as hi tologically obsen-ed (Fig. -I). Our diagnosis \\-as based on light micrascopy. The pathologie findings of the case presented here are typical of maJignant nen-e sheath tumors (Fig . 1, 2, 3). Although it simulated schwannoma in some aspects, the lesion was more cellular, had prominant abnormal mitotic figures, and showed an infiltrating growth into the thyraid, muscle tissue and vessels_
Fig. 2 Well-developed nuclear schwannoma (H E x 140)
Fig. 3
palisading
Whorled structure (H E x 700)
within
the
malignant
In maJignant sch\\-annoma of more than 5 cm in diameter, and the presence of neurofibramatosi sunrjvaJ is greaUy decreased. Patients with tumor in the extremities do better than those with head and neck lesions. 1 0 significant correlation has been noted between sumval and either grade or mitotic rate. Adjuvant radiation or chemotherapy does not appear to affect sumvaJ of maJignant schwannoma which is an aggressive, uncommon neoplasm and large tumor ize, the presence of neurofibromatosis and total resection are the most important prognostic indicators (3-6, 9, 10, 12). Orten they invade critical structures, in such cases the resection has to be limited, and this limitation contributes to poor prognosis. The majority of the
Downloaded by: Universite de Sherbrooke. Copyrighted material.
228
Eur J Pediatr Surg 2 (1992)
229
patients die wilhin two and a half years after the diagnosis is made (17).
European Journal of Pediatric Surgery
Chance 01 Survival: Good!
In our case, a wide surgical resection was the preferred method of treatment \vith postoperative chemotherapy.
Many complicated operations on children - such as successful separation of Siamese twins have only recently become possible thanks to advances in surgery and postoperative aftercare. European
References Dicurlo EF, II'oodnlff j,lI, Bansal .11. Erlandson RA.' The purely epilhelioid malignanl peripheral ner',e sheath tumor. Am J Surg Pathol 10 (1986) 178-·190 2 DII«/Iman BS. Scheilhaller BI\': Postirradialion neurofibrosarcoma. Cancer 51 (198:.l) 1028-1 O:.l:.l 3 DII«/Iman BS. Schei/haller 13\\'. Piepgras DG. Reiman /1.11. flslllP D.I/ .\\alignant peripheral ner\'e sheath tumors, a e1inicopalhologie stuel,' of 120 eases. Cancer 57 (1986) 2006-2021 ·1 Ddnger F.!l. \\'eiss SI\': Soft Tissue Tumors. The C. \. \\osh.l' Compa m. SI. Louis \\'ashington D.C. Toronto (1 BHH) 781-H 15 5 Ge/llla/as CS, Exarhako, G. "·ondi·Patlli .1, I\amlas 1)... I/hana"", C, I'oliti IID: .\\alignanl sehllannoma of the slomaeh in a patienl 11 ilh neurofibromalosis. Eur J Surg Oneol I I (I BHH) 261-261 ö (,IllIane P). Gilber/ RII". ,',111 ,\'o,/r,/n" . I I\P, Slinga RP \\align schl\;lJ1noma in the head and neck. J Otolar, ngol I 1 (I 9H,-») 171-17,') ~ 1"1/"11 SI. Diffel'ential DIagnosis of Soft Ti"ue ancll30ne Tumors. Lead ('ehiger, Philadelphia 1~IH6. p. :lO. 38. 59. II(i. 118. 151.201. 2:.l6. 27:j. 271. :HJ2 H I"/rkin). Reed Rj Tumors of lhe Peripheral '\enous Syslem. In: Alias of Tumor Palhology. seeonel se ries faseiele :J. ,\rmed Fon'es Institule of I';, lhology \\'ashinglon D.C. (1 B6D) 10/-1 :16 9/1111
Acute Respiratory Distress Due to Malignant Schwannoma. A Case Report M. MeUkoglu J, S. KaravelL'2, T (;olak J, T Gelen2 Departments of I Pediatric Surgery and 2pathology, Medical Faculty, UniversiLy of Akdeniz, AnLalya, Turkey
A 13-year-old boy with acute re piratory distress due to a malignant schwannoma located in the neck, is reported. This case suggested that maJignant schwannoma in the neck can lead to airway obstruction.
Key words
1alignant schwannoma - Malignant primar}' nen'e sheath tumors - Acute respiratory distress
Resume
Les auteurs rapportent l'observation d'un gan;on de 13 ans atteint d'une detresse respiratoire aigue par schwann6me malin localise au niveau du cou. Ce cas suggere que les schwann6mes malins dans le cou peuvent conduire a une obstruction des voies respiratoires.
Mols-eIes
Schwann6me malin - Tumeur maligne primitive de couverture nerveuse - Detresse respiratoire aigue
Zusammenfassung
Ein 13jähriger Junge mit einem malignen Schwannoma im Halsbereich, das zu akuten Atemstörungen führte, wird vorgestellt.
l- - - Schlüsselwörter
Malignes Schwannom - Akute Atemnot
Extracranial neurogenie tumors of the head and neck are relatively rare tumors that are often deceptive and may be mistaken for other disease processes (3, 4, 6, 7, 9, 12, 14, 16, 17). Malignant nerve sheath tumors arise from Schwann cells of neuroectodermal origin, and from fibrocytes of mesodermal origin (3,4,5,7,8,10,11,15). Approximately 6-16 % of malignant schwannomas occur in the head and neck (6, 14, 17). ~10st often they arise from cranial nen'es, upper branches of the brachial plexus, cenrlcal nen'e rools and sympathetic chain. Lymph node metastases are rare but local recurrence and hematogenous spread to the lungs are not uncommon. Treatment of malignant schwannoma involves radicallocal excision as the primary mode of treatment (3, 14, 16, 17). Overa1l5-year survivals have varied from 22-30 % (6).
Case report A 13-year-old bay (B. B.) was admitted to the hospital on September 16, 1988, with acute respiratory distress. He had a six months' history of a rapidly enlarging mass on the mid-front of the neck and increasing respiratory distress during the last fifteen days. On physical examination: he was an ill-looking and conscious boy with respiratory distress. He had minimal cyanosis and mild respiratory distress with supraclavicuJar, substernal and intercostal retractions. There was a 10 x 10 x 5 cm fixed mass on the mid front of neck \vith rubber consistency. The overlying skin of the mass was normal. There was no cervieal, axillary and supraclavicuJar lymphadenopathy. The respiration sounds were found to be decreased at the lower lobes. The heart sounds were normal. In direct laryngoscopy, the voeal cords were normal. The rest of the physieal examination findings was normal. Since he had severe respiratory distress, an emergency operation was decided. At the operation, a necrotic and ruhbery mass of 10 x 8 x 5 cm diameter over the trachea, displacing the lobes of the thyroid, was observed. The mass was resected totally, and a tracheotomy was performed due to the risk of tracheomalacia due Lo compression of the trachea by the tumor mass. The postoperative period was uneventfuJ.
Pathologie findings Recei\ed \ larch 11, 1991 Eur J Pediatr Surg 2 (J 992) 227-229 © Hippokrates \'erlag Stuttgart ,\Iasson Editeur Paris
Gross specimens from the original operation included fragments of friable pink-white tissue, firm to soft, depending on the ab ence or presence of necrosis and haemorrhage. .'vlicroscopical examination of the original specimen showed that the tumor was not truly en-
Downloaded by: Universite de Sherbrooke. Copyrighted material.
Introduction
Summary
Eur J Pediatr urg 2 (1992)
,\1. Meltkoglu et al
eapsulated and in\"aded the th}Toid tissue and surrounding muscle tissue and vessels. The pathologie findings of the ease are typieal of the malignant nerve sheath tumors (Figs. 1,2,3).
Clinical course On the 13th postoperative day the patient was sent horne in excellent eondition. SubsequenUy, the patient underwent two courses of Adriablastine, Endoxan and \'ineristine ehemotherapy. He was followed up at monthly inten·al \\ith computerized tomography and found to be normal. _ 0 reeurrent tumor growth and metastases could be identified. He has been aS}Tnptomatie for a one year follow-up period.
Discussion
The patient reported herein represents a case of malignant sch\\-annoma in the neck. Acute respiratol}' distress due to malignant sch\\"annoma in the neck has been reported before (6, 9, l-l, 16). Our ca e is under the age of 20 years and there is no association \\-ith von Recklinghausen's neurofibromatosis.
Fig. 1 The mallgnant x1400
~chwa~nOfT1a
show ng abnormal mltoses (>-I E
[n our case, the tumor mass appeared to be encapsulated, but lhis pseudo-encapsulation \\'as secondal}' to compression of surrounding structures and infiltration of epineurium ,,-as hi tologically obsen-ed (Fig. -I). Our diagnosis \\-as based on light micrascopy. The pathologie findings of the case presented here are typical of maJignant nen-e sheath tumors (Fig . 1, 2, 3). Although it simulated schwannoma in some aspects, the lesion was more cellular, had prominant abnormal mitotic figures, and showed an infiltrating growth into the thyraid, muscle tissue and vessels_
Fig. 2 Well-developed nuclear schwannoma (H E x 140)
Fig. 3
palisading
Whorled structure (H E x 700)
within
the
malignant
In maJignant sch\\-annoma of more than 5 cm in diameter, and the presence of neurofibramatosi sunrjvaJ is greaUy decreased. Patients with tumor in the extremities do better than those with head and neck lesions. 1 0 significant correlation has been noted between sumval and either grade or mitotic rate. Adjuvant radiation or chemotherapy does not appear to affect sumvaJ of maJignant schwannoma which is an aggressive, uncommon neoplasm and large tumor ize, the presence of neurofibromatosis and total resection are the most important prognostic indicators (3-6, 9, 10, 12). Orten they invade critical structures, in such cases the resection has to be limited, and this limitation contributes to poor prognosis. The majority of the
Downloaded by: Universite de Sherbrooke. Copyrighted material.
228
Eur J Pediatr Surg 2 (1992)
229
patients die wilhin two and a half years after the diagnosis is made (17).
European Journal of Pediatric Surgery
Chance 01 Survival: Good!
In our case, a wide surgical resection was the preferred method of treatment \vith postoperative chemotherapy.
Many complicated operations on children - such as successful separation of Siamese twins have only recently become possible thanks to advances in surgery and postoperative aftercare. European
References Dicurlo EF, II'oodnlff j,lI, Bansal .11. Erlandson RA.' The purely epilhelioid malignanl peripheral ner',e sheath tumor. Am J Surg Pathol 10 (1986) 178-·190 2 DII«/Iman BS. Scheilhaller BI\': Postirradialion neurofibrosarcoma. Cancer 51 (198:.l) 1028-1 O:.l:.l 3 DII«/Iman BS. Schei/haller 13\\'. Piepgras DG. Reiman /1.11. flslllP D.I/ .\\alignant peripheral ner\'e sheath tumors, a e1inicopalhologie stuel,' of 120 eases. Cancer 57 (1986) 2006-2021 ·1 Ddnger F.!l. \\'eiss SI\': Soft Tissue Tumors. The C. \. \\osh.l' Compa m. SI. Louis \\'ashington D.C. Toronto (1 BHH) 781-H 15 5 Ge/llla/as CS, Exarhako, G. "·ondi·Patlli .1, I\amlas 1)... I/hana"", C, I'oliti IID: .\\alignanl sehllannoma of the slomaeh in a patienl 11 ilh neurofibromalosis. Eur J Surg Oneol I I (I BHH) 261-261 ö (,IllIane P). Gilber/ RII". ,',111 ,\'o,/r,/n" . I I\P, Slinga RP \\align schl\;lJ1noma in the head and neck. J Otolar, ngol I 1 (I 9H,-») 171-17,') ~ 1"1/"11 SI. Diffel'ential DIagnosis of Soft Ti"ue ancll30ne Tumors. Lead ('ehiger, Philadelphia 1~IH6. p. :lO. 38. 59. II(i. 118. 151.201. 2:.l6. 27:j. 271. :HJ2 H I"/rkin). Reed Rj Tumors of lhe Peripheral '\enous Syslem. In: Alias of Tumor Palhology. seeonel se ries faseiele :J. ,\rmed Fon'es Institule of I';, lhology \\'ashinglon D.C. (1 B6D) 10/-1 :16 9/1111
