Malignant Triton Tumor without Von Recklinghausen’s Neurofibromatosis in a 16 year-old Indonesian Girl: A Case Report

Background: To diagnose malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation in malignant triton tumor which has a rare incidence. Case Illustration: We reported a case in a 16 year-old girl who presented with progressively painless proptosis of the left eye and decreased visual acuity. There was no evidence of Recklinghausen’s disease. She underwent surgery and radiotherapy. The histopatologic findings showed malignant peripheral nerve sheath tumor (MPNST). Four years later, she suffered recurrence proptosis and decreased visual acuity. Histomorphology supported by immunostaining with S-100 protein confirmed the diagnosis of malignant triton tumor. She underwent chemotherapy and enucleation. Conclusion: This case highlights the prudent use of immunohistochemistry that is essential in making an early detection and a correct diagnosis.

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Malignant Peripheral Nerve Sheath Tumor With Rhabdomyosarcomatous Differentiation (Malignant Triton Tumor)

Archives of Pathology & Laboratory Medicine ◽

10.5858/2006-130-1878-mpnstw ◽

2006 ◽

Vol 130 (12) ◽

pp. 1878-1881 ◽

Cited By ~ 5

Author(s):

Christopher J. Stasik ◽

Ossama Tawfik

Keyword(s):

Peripheral Nerve ◽

Correct Diagnosis ◽

Strong Association ◽

Clinical History ◽

Nerve Sheath Tumor ◽

Malignant Triton Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Prognostic Features ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

Abstract Malignant peripheral nerve sheath tumors arise from Schwann cells or within existing neurofibromas and have a strong association with type 1 neurofibromatosis. These tumors are histologically diverse and may contain malignant areas of divergent mesenchymal differentiation, the most common of which is skeletal muscle (rhabdomyosarcoma). Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation is also known as malignant triton tumor. Malignant triton tumor has a worse prognosis than classic malignant peripheral nerve sheath tumor does, and the correct diagnosis requires attention to the clinical history and knowledge of the complexities regarding its differential diagnosis. In this review we discuss the clinical, histopathological, immunohistochemical, and prognostic features of this rare neoplasm.

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Case report and immunohistochemical study of a malignant peripheral nerve sheath tumor in a dog

Brazilian Journal of Veterinary Research and Animal Science ◽

10.11606/issn.1678-4456.bjvras.2017.132447 ◽

2017 ◽

Vol 54 (4) ◽

pp. 420

Author(s):

Thaís De Almeida Moreira ◽

Leandro Willian Borges ◽

Talita Lopes Serra ◽

Diego Petrocino Caetano ◽

Marcelo Carrijo da Costa ◽

...

Keyword(s):

Case Report ◽

Peripheral Nerve ◽

Immunohistochemical Study ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath ◽

S 100

O tumor maligno da bainha do nervo periférico é raro e pouco descrito em cães. Classicamente, o termo “neurofibroma/sarcoma” é empregado quando o tumor é composto por células de Schwann e células perineurais. Neste relato são descritos os achados clínicos, histopatológicos e imuno-histoquímicos de um caso de tumor maligno da bainha do nervo periférico, provavelmente neurofibrossarcoma, localizado no subcutâneo do membro pélvico de um cão da raça Rottweiler. Os achados histopatológicos associados às observações imuno-histoquímicas contribuíram para o diagnóstico, sendo observada positividade para proteína S-100, vimentina e CD57, permitindo a diferenciação da neoplasia em questão de outros tumores malignos com características morfológicas similares.

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A rare case of retroperitoneal malignant triton tumor invading renal vein and small intestine

Vojnosanitetski pregled ◽

10.2298/vsp1303322m ◽

2013 ◽

Vol 70 (3) ◽

pp. 322-325 ◽

Cited By ~ 1

Author(s):

Zaklina Mijovic ◽

Dragan Mihailovic ◽

Nikola Zivkovic ◽

Milos Kostov ◽

Sladjana Zivkovic ◽

...

Keyword(s):

Small Intestine ◽

Peripheral Nerve ◽

Renal Vein ◽

Correct Diagnosis ◽

Late Complication ◽

Vena Cava ◽

Nerve Sheath Tumor ◽

Malignant Triton Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath

Introduction. Malignant Triton tumor is a very rare malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation. Most of those tumors occur in patients with von Recklinghausen?s disease or as a late complication of irradiation and commonly seen in the head, neck, extremities and trunk. Case report. We reported retroperitoneal malignant Triton tumor in a 57-year-old female patient. Skin lesions were not present, and there was no family history of neurofibromatosis or previous irradiation. The presented case is one of a few recorded in the specialized literature that occurs in the retroperitoneal space in sporadic form. In this case, tumor consisted of a multilobular mass was in close relation with the abdominal aorta and inferior vena cava and involved the renal vein with gross invasion of the small intestine. The patient underwent total resection of the tumor and left nefrectomy was performed. The small intestine 10 cm in length was also resected and end-to-end anastomosis was conducted. The postoperative course was uneventful and the patient was discharged from the hospital ten days after the surgery. Conclusion. Diagnostically, it is crucial to recognize this uncommon histological variant because malignant Triton tumor has a worse prognosis than classic malignant peripheral nerve sheath tumor does. The use of the immunohistochemistry is essential in making the correct diagnosis. Only appropriate pathological evaluation supported by immunostaining with S-100 protein and desmin confirmed the diagnosis. Aggressive surgical management treatment improves the prognosis of such cases with adjuvant radiotherapy.

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Malignant peripheral nerve sheath tumor of the orbit: a case report and review of the literature

Orbit ◽

10.1080/01676830.2021.1918178 ◽

2021 ◽

pp. 1-5

Author(s):

Kara E. Jones ◽

Ami Patel ◽

Mary G. Kunesh ◽

Bradley A. Thuro

Keyword(s):

Case Report ◽

Peripheral Nerve ◽

Nerve Sheath Tumor ◽

Review Of The Literature ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath

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RARE-20. MALIGNANT PERIPHERAL NERVE SHEATH TUMOR OF A CRANIAL NERVE IN AN INFANT WITH NEUROCUTANEOUS MELANOSIS

Neuro-Oncology ◽

10.1093/neuonc/noaa222.731 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii446-iii446

Author(s):

Lacey Carter ◽

Naina Gross ◽

Rene McNall-Knapp ◽

,and Jo Elle Peterson

Keyword(s):

Peripheral Nerve ◽

Cranial Nerve ◽

Leptomeningeal Disease ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Neurocutaneous Melanosis ◽

Spindle Cell Neoplasm ◽

Lower Back ◽

Nerve Sheath ◽

S 100

Abstract At one month of age, a female presented with a giant congenital nevus along lower back and thighs and hydrocephalus. A ventriculoperitoneal shunt was placed. An MRI was done at six months, initially reported as normal. At eleven months of age, five months after original MRI, patient presented with dysconjugate gaze and lethargy. MRI showed new 3.8 x 3.7 x 3.4 cm right cerebellopontine angle mass extending into Meckel’s cave and foramen ovale along with leptomeningeal disease extending from the mass along the entire length of the spinal cord. Retrospective review of prior MRI revealed subtle leptomeningeal enhancement concerning for neurocutaneous melanosis (NCM). Given the leptomeningeal disease, family elected for open biopsy and debulking of lesion instead of aggressive resection. Histologically, the mass showed hypercellular spindle cell neoplasm with mitotic activity and necrosis mixed with remnants of normal cranial nerve. GFAP was negative, excluding a glioma. HMB-45, MITF, panmelanoma, and Melan-A were negative, excluding melanoma. A negative myogenin stain ruled out ectomesenchymoma. S-100 protein and SOX-10 positivity with variable loss of staining for trimethylation of histone H3 K27 were indicative of malignant peripheral nerve sheath tumor (MPNST). Given the course of the mass, trigeminal nerve MPNST was presumed. Given the poor prognosis of intracranial MPNST and NCM, family elected to forgo treatment and was discharged with hospice. She died 25 days after surgery. Cranial nerve MPNST is rare. MPNST in patients with NCM has not previously been reported to our knowledge.

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