Tumor tritón maligno en región mandibular y cervical / Malignant triton tumor in the mandibular and cervical region

Introducción: El tumor tritón maligno es una neoplasia rara en la que se encuentran células rabdomioblásticas en un tumor maligno de la vaina de nervios periféricos, que se caracteriza por su agresividad y mal pronóstico. La localización en la cabeza y el cuello es poco frecuente. La inmunohistoquímica juega un papel importante en el diagnóstico. Objetivo: Describir un tumor tritón maligno de tamaño inusual. Presentación del caso: Paciente femenino, de 16 años, es referida al servicio de cirugía maxilofacial del Instituto Nacional de Pediatría, Ciudad de México, con un diagnóstico de tumor neuroectodérmico en región facial y cervical de un año de evolución. Clínicamente el tumor era exofítico, multilobulado, con zonas extensas de necrosis, superficie de varias tonalidades y un tamaño aproximado de 18 x 10 x 12 cm. Se realizó una biopsia e inmunohistoquímica que confirmó el diagnóstico de tumor tritón maligno. La paciente fue intervenida quirúrgicamente, procedimiento con el cual se eliminó totalmente la lesión, con márgenes de seguridad. La paciente presentó una evolución tórpida, con desenlace fatal al cabo de seis meses del tratamiento. Conclusiones: El tumor tritón es una neoplasia agresiva y su detección oportuna orienta al cirujano a ofrecer al paciente un tratamiento adecuado(AU)

Introduction: Malignant triton tumor is a rare neoplasm in which rhabdomyoblasts are present in a malignant tumor of the peripheral nerve sheath. This condition is characterized by its aggressiveness and bad prognosis. Location in the head and neck is infrequent. Immunohistochemical testing plays an important role in its diagnosis. Objective: Describe an unusually large malignant triton tumor. Case presentation: A case is presented of a female 16-year-old patient referred to the maxillofacial surgery service of the National Institute of Pediatrics in Mexico City with a diagnosis of neuroectodermal tumor of one year's evolution in the facial and cervical region. In clinical terms, the tumor was exophytic, multilobed, with extensive areas of necrosis, a surface in several shades of color and an approximate size of 18 x 10 x 12 cm. Biopsy and immunohistochemical testing confirmed the diagnosis of malignant triton tumor. The patient underwent surgery in which the lesion was totally excised with a safety margin. Evolution was clumsy, with a fatal outcome at six months of treatment. Conclusions: Triton tumor is an aggressive neoplasm whose early detection makes it possible for surgeons to provide an appropriate treatment(AU)

Malignant Triton tumor: a rare cause of sciatic pain and foot drop / Tumor de Triton maligno: uma causa rara de dor ciática e pé pendente

ABSTRACT Malignant peripheral nerve sheath tumors (MPNST) are very rare and are frequently localized in the buttocks, thigh, arm, or paraspinal region; one variant is the malignant Triton tumor, with rhabdomyosarcomatous differentiation. The authors present a challenging differential diagnosis of a sciatic pain and foot drop in a woman with history of lumbar disk herniation, which was found to be caused by a Triton tumor of the sciatic nerve. She underwent surgical excision, followed by radiation and chemotherapy. Malignant Triton tumor cases have rarely been described and reported in the literature. The recommended treatment is radical excision followed by high-dose radiotherapy and chemotherapy. The prognosis, although poor, depends on the location, grade, and completeness of surgical margins.

RESUMO Os tumores malignos da bainha dos nervos periféricos (TMBNP) são muito raros e localizam-se mais frequentemente na região nadegueira, paraespinal, coxa ou braço; uma variante é o tumor de Triton maligno, com uma diferenciação rabdomiosarcomatosa. Apresentamos um diagnóstico diferencial desafiante de dor ciática e pé pendente em uma paciente com antecedentes de hérnia discal lombar, que se descobriu que era causada por um tumor de Triton do nervo ciático. A paciente foi submetida a excisão cirúrgica, seguida de radio e quimioterapia. Poucos casos de tumores de Triton malignos foram descritos e relatados na literatura. O tratamento recomendado é a excisão radical, seguida de radioterapia em alta dose e quimioterapia. O prognóstico, embora mau, depende da localização, do grau e das margens cirúrgicas da exérese.

Tumor de tritón benigno: reporte de un caso en órbita / Orbit Benign triton Tumor: Case Report

El artículo presenta el caso de una mujer de 49 años de edad sin antecedentes patológicos con proptosis axial progresiva izquierda de un año de evolución asociada a dolor ocular, sin cambios en la agudeza visual, sin limitación en los movimientos oculares ni diplopía. Con imágenes sugestivas de seudotumor versus schwannoma versus hemangioma cavernoso orbitario versus dermoide intraconal. Fue llevada a resección de masa vía orbitotomía anterior transconjuntival. Esta fue una lesión compatible histológicamente con hamartoma neuromuscular (tumor de tritón benigno).

This article shows the case of a 49-year-old woman with no medical history, developed a progressive left axial proptosis associated with ocular pain. There are no changes to visual acuity, limitation of eye movement or diplopia. The diagnostic images may suggest one of the following: pseudotumor, schwannoma, orbital cavernous hemangioma or intraconal dermoid. The resected specimen was taken via anterior transconjuntival orbitotomy. Microscopic examination of the tumor showed a neuromuscular hamartoma (benign triton tumor).

Tumor maligno de tritón pélvico: reporte de un caso / Pelvic triton malignant tumor: case report

Se presenta el caso de una paciente de 29 años con dolor en la región glútea y miembro inferior derecho, portadora de una enfermedad de Von Recklinghausen y que desarrolló dos tumoraciones pélvicas retroperitoneales de crecimiento rápido. En la laparotomía se encontró las tumoraciones adheridas al hueco obturatriz y alerón del hueso iliaco. Se procedió a exéresis de las tumoraciones, la del lado derecho de mayor tamaño, de evolución rápida y compatible con tumor de Tritón, y la izquierda compatible con neurofibroma.

We report the case of a 29 year-old woman complaining of pain in right buttock and leg. She suffered of von Recklinghausen disease and developed rapidly growing retroperitoneal pelvic tumors. At laparotomy the tumors were attached to the obturator foramen and the iliac wing. The larger and fast growing right side tumor compatible with malignant triton tumor, and the left sided neurofibroma were removed.

Adulthood Benign Triton Tumor Developed in the Orbit

Benign triton tumor (BTT) or neuromuscular hamartoma is an uncommon tumor composed of mature neural and well-differentiated striated muscular elements. Its development is exceptionally rare in the adult and head region. This report describes a case of adulthood BTT that occurred in the orbit. The patient was a 53-year-old woman who presented with right periorbital swelling and pain in eyeball over 2 months. Magnetic resonance imaging revealed a well-enhancing mass surrounding optic nerve and ocular muscles in the right retrobulbar area. The tumor was subtotally removed via transcranial approach. Its pathological diagnosis was confirmed to be a neuromuscular hamartoma. She developed diplopia postoperatively. Adulthood BTT should be considered in the differential diagnosis of head and neck tumors. It is also important to make adequate therapeutic strategy to avoid postoperative neural dysfunction.

Benign Triton Tumor: A Rare Entity in Head and Neck Region

Benign triton tumors (BTT) are very rare lesions composed of mature skeletal muscle and neural tissue. We report a case of a 14-year-old boy who presented with asymptomatic swelling of the chin over an 18-month duration which increased gradually to involve the left side of the lower lip. Clinically, a diagnosis of neurofibroma was made. Excisional biopsy confirmed the diagnosis of a BTT. Having an affinity for large nerve trunks like the brachial and sciatic, these tumors rarely occur in the head and neck region. When they do, they may involve the large central cranial nerve trunk and present as intracranial masses or involve the smaller peripheral nerve twigs and present as asymptomatic skin nodules, of which only four cases involving peripheral nerves are reported in the English literature. Here, we report the fifth documented case of a BTT involving the mental branch of the trigeminal nerve. A brief review of the literature is also provided.

Tumor maligno da bainha neural periférica com diferenciação rabdomioblástica (tumor de triton maligno) pós-radioterapia / Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation ("malignant triton tumor") after radiation therapy

É relatado o primeiro caso de tumor maligno de bainha neural periférica com diferenciação rabdomioblástica (tumor de triton maligno), induzido por radioterapia por câncer de próstata com comprometimento (metástase) dos nódulos linfáticos localizados na região pélvica, com curto período de latência e de péssima evolução clínica. O paciente foi a óbito, sendo realizado exame necroscópico que revelou metástases para pulmões, fígado, diafragma e mesocólon.

We report the first case of a malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation ("triton tumor") induced by radiotherapy for prostate cancer with pelvic lymph node metastasis, with a short latency period and poor clinical outcome. The patient died and necropsy revealed multiple metastases in the lungs, liver, diaphragm and mesocolon.

Clinicopathological analysis of malignant triton tumor of the posterior mediastinum / 上海第二医科大学学报

Objective To investigate the pathologic appearances,immunohistochemical features,and genetic changes of malignant triton tumor(MTT). Methods One case of MTT was studied pathologically and immunohistochemically,and the related literatures were reviewed. Results A huge mass,demonstrated in the thorax by X ray and CT scan was seen in the posterior mediastinum in the surgery.Histologically,the tumor was composed of spindle cells with significant atypia.Some of the tumor cells had dense eosinophilic cytoplasm.Immunohistochemical staining revealed positive for myoglobin,desmin and S-100 in most of the tumor cells.The pathological diagnosis was MTT of the posterior mediastinum. Conclusion Cases of MTT in the mediastinum are very rare,with less specific clinical and imaging manifestations.The diagnosis is mainly made on the basis of pathological examination and immunohistochemical staining.

CT and MRI Manifestations of Primary Malignant Triton Tumor:A Report of 3 Cases with Review of the Literature / 实用放射学杂志

Objective To improve the understanding of malignant triton tumor on imaging findings.Methods CT and MRI findings of rare malignant triton tumor in 3 cases confirmed histologically were analysed with review of the literatures.Results 3 tumors included prostatic malignant triton tumor with pulmonary metastatic tumors and neurofibromatosis-I in 1 case,left haunch malignant triton tumor in 1 case and right maxillary sinus malignant triton tumor with destruction of maxillary sinus paries in 1 case.The imaging appearances of tumors were the huge soft tissue mass which strongly suggested malignant tumors,high signal intensity on T2WI,circular septa inside the tumors with low signal intensity.Conclusion Although the qualitative diagnosis of malignant triton tumor depends on pathological examination,CT and MRI play a useful role in diagnosis and differential diagnosis of this tumors.

Subcutaneous Neuromuscular Hamartoma: A case report

Subcutaneous form of neuromuscular hamartoma is extremely rare and histologically different from the conventional neuromuscular hamartoma of the peripheral nerve or benign Triton tumor by an indistinct nodular growth with ill-defined margin and marked collagen interposition. It is usually not associated with a major nerve. We report a case of subcutaneous neuromuscular hamartoma developed in the forehead of 24-year-old man. The tumor showed proliferation of dense, hyalinized fibrous tissue, in which single or group of mature skeletal muscle fibers and nerve fibers were haphazardly intermixed. Recognition of abnormally arranged muscle and nerve fibers is important not to miss this lesion.

A Case of Malignant Triton Tumor In Nasal Cavity / 대한이비인후과학회지

Malignant triton tumor is a rare and usually aggressive sarcoma consisting of a malignant schwannoma with rhabdomyoblastic differentiation. Although this tumor is usually found at the extremities, 20% of cases are located in the head and neck region. This unusual tumor has not yet been described in the Korean Journal of Otolaryngology-Head and Neck Surgery. Malignant triton tumor is histologically high-grade, with large numbers of mitoses, prominent necrosis, and cellular pleomorphism. The clinical course for malignant triton tumor is usually one of rapid growth, early metastasis, and poor outcome in spite of therapy. Recently we experienced a case of malignant triton tumor, which involved the nasal cavity, maxillary and ethmoid sinus. The malignant tumor was treated with wide surgical excision followed by radiation therapy.