Tumor maligno de la vaina del nervio periférico del ciático izquierdo

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Abstract

RESUMEN Fundamento: los tumores malignos de la vaina del nervio periférico, son sarcomas de partes blandas con una baja incidencia. Objetivo: presentar un caso de tumor maligno de la vaina del nervio ciático izquierdo que recibió tratamiento quirúrgico en el Hospital Universitario Manuel Ascunce Domenech de la provincia Camagüey en junio de 2018. Presentación del caso: paciente masculino de 45 años, antecedentes de neurofibromatosis tipo I. Historia de dolor ciático troncular izquierdo que se hizo constante. Refirió dificultad para la marcha y aumento de volumen a nivel del glúteo izquierdo. Se palpó tumoración de siete centímetros de diámetro de superficie lisa, firme, fija a planos profundos y dolorosos a la manipulación. La ultrasonografía de partes blandas mostró: imagen compleja de localización profunda hipoecóica ovalada de 45 X 9 X 20 milímetros, de contornos regulares, bien definidos a una profundidad de la piel de 22 mm. Se decidió tratamiento quirúrgico basado en la excéresis de la tumoración. La biopsia definitiva luego de la inmunohistoquímica informó: tumor maligno de la vaina del nervio periférico de alto grado en un neurofibroma previo. Conclusiones: los tumores malignos de la vaina del nervio ciático son neoplasias raras y aún más su localización proximal. En el caso que se presentó a pesar de su resección quirúrgica radical asociada a radioterapia, el tamaño superior a diez centímetros, la asociación a la neurofibromatosis tipo I y el alto grado histológico, ensombrecen el pronóstico. Es importante el diagnóstico precoz y tratamiento oportuno.

Translated abstract

ABSTRACT Background: malignant peripheral nerve sheath tumors are soft tissue sarcomas with a low incidence. Objective: to present a case of malignant peripheral nerve sheath tumor of the left sciatic nerve who underwent surgical treatment at the University Hospital Manuel Ascunce Domenech in the province of Camagüey in June, 2018. Presentation of the case: a 45-year-old male patient, personal history of neurofibromatosis type 1 with a truncal left sciatic pain that turned permanent. He referred gait difficulties and increase of the volume in the left buttock. A tumor mass of seven centimeters was palpated, firm, fix to deep planes and painful at deep palpation. Soft tissue ultrasonography showed: complex oval hypoechoic image of deep location of 45 X 9 X 20 millimeters of regular and well defined contours at 22 millimeters depth from the skin. Surgical treatment was decided base on the tumor resection and radiotherapy. Definitive biopsy after immune histochemistry, informed a high degree malignant peripheral nerve sheath tumor of a previous neurofibroma. Conclusions: malignant peripheral nerve sheath tumors are weird neoplasias and even more its proximal location. The presented patient despite the radical surgical resection associated to radiotherapy, the size superior to ten centimeters, the association to neurofibromatosis type 1 and the high histological degree, worsen the forecast. The precocious diagnosis and accurate treatment are important.

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Malignant peripheral nerve sheath tumors.

Mohamad Farid, Elizabeth Demicco, Roberto Marín García … (2014)

Malignant peripheral nerve sheath tumors (MPNST) are uncommon, biologically aggressive soft tissue sarcomas of neural origin that pose tremendous challenges to effective therapy. In 50% of cases, they occur in the context of neurofibromatosis type I, characterized by loss of function mutations to the tumor suppressor neurofibromin; the remainder arise sporadically or following radiation therapy. Prognosis is generally poor, with high rates of relapse following multimodality therapy in early disease, low response rates to cytotoxic chemotherapy in advanced disease, and propensity for rapid disease progression and high mortality. The last few years have seen an explosion in data surrounding the potential molecular drivers and targets for therapy above and beyond neurofibromin loss. These data span multiple nodes at various levels of cellular control, including major signal transduction pathways, angiogenesis, apoptosis, mitosis, and epigenetics. These include classical cancer-driving genetic aberrations such as TP53 and phosphatase and tensin homolog (PTEN) loss of function, and upregulation of mitogen-activated protein kinase (MAPK) and (mechanistic) target of rapamycin (TOR) pathways, as well as less ubiquitous molecular abnormalities involving inhibitors of apoptosis proteins, aurora kinases, and the Wingless/int (Wnt) signaling pathway. We review the current understanding of MPNST biology, current best practices of management, and recent research developments in this disease, with a view to informing future advancements in patient care.

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Cancer of the Peripheral Nerve in Neurofibromatosis Type 1

Verena Staedtke, Ren-Yuan Bai, Jaishri O'Neill Blakeley (2017)

The RASopathy neurofibromatosis 1 is an autosomal dominant hereditary cancer syndrome that represents a major risk for the development of malignancies, particularly malignant peripheral nerve sheath tumors (MPNSTs). MPNSTs are unique sarcomas that originate from the peripheral nerve and represent the only primary cancer of the peripheral nervous system. To date, surgery is the only treatment modality proven to have survival benefit for MPNSTs and even when maximal surgery is feasible, these tumors are rarely curable, despite the use of chemotherapy and radiation. In this review, we discuss the current state-of-the-art treatments for MPNSTs, latest therapeutic developments, and critical aspects of the underlying molecular and pathophysiology that appear promising for therapeutic developments in the future. In particular, we discuss the specific elements of cancer in the peripheral nerve and how that may impel development of unique therapies for this form of sarcoma. Electronic supplementary material The online version of this article (doi:10.1007/s13311-017-0518-y) contains supplementary material, which is available to authorized users.

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Giant intrapelvic malignant peripheral nerve sheath tumor mimicking disc herniation: A case report

Peng Wang, Cong Chen, Xiaotang Xin … (2016)

Giant intrapelvic malignant peripheral nerve sheath tumors arising in the sciatic nerve in the pelvic cavity are a rare occurrence and their symptomatology is usually misdiagnosed as intervertebral disc herniation. We herein report the case of a 46-year old woman presenting with pain, hypesthesia and weakness of the left lower extremity due to a giant intrapelvic malignant peripheral nerve sheath tumor of the sciatic nerve. Prior to being referred to our institution, the patient was misdiagnosed as a case of sciatica due to a lumbar disc herniation and underwent an operation unsuccessfully, as there was little symptomatic improvement 2 months after the surgery. A magnetic resonance imaging examination of the pelvic cavity revealed a tumor of the sciatic nerve. The mass was resected via the posterior approach and histopathological examination confirmed the diagnosis of malignant peripheral nerve sheath tumor. Intrapelvic malignant peripheral nerve sheath tumors are an uncommon cause of sciatica and are commonly misdiagnosed as lumbar intervertebral disc herniation. Accurate diagnosis and complete surgical excision prior to metastasis are crucial for effective management of this condition.