Fibroblastic Malignant Peripheral Nerve Sheath Tumour of the Uterine Cervix: Report of a Case and Literature Review With Emphasis on Possible Differential Diagnosis.

07:00 EST 22nd February 2018 | BioPortfolio

Summary of "Fibroblastic Malignant Peripheral Nerve Sheath Tumour of the Uterine Cervix: Report of a Case and Literature Review With Emphasis on Possible Differential Diagnosis."

Cervical sarcomas are rare neoplasms, accounting for <1% of all cervical malignancies and characterized by an aggressive course despite radical excision. We report the clinical and microscopic features of a spindle cell sarcoma arising as a polypoid endocervical mass in a 45-yr-old woman. The neoplasm was characterized by a monotonous, mildly atypical proliferation of spindle cells, displaying a fibrosarcoma-like parallel pattern of highly dense fascicles, growing under the cervical epithelium. Mitotic activity was conspicuous, with up to 40 mitoses per 10 HPF. On immunohistochemistry, tumor cells were patchy S-100 protein positive. Additional immunohistochemical markers performed to rule out smooth muscle, melanocytic, epithelial, and sarcomatous differentiation were negative. A possible monophasic synovial sarcoma was also excluded by negative fluorescence in situ hybridization t(X;18) analysis. Interestingly, the neoplasm showed a focal CD34 positivity, as reported in normal fibrocytic cells of the endocervical stroma. Giving the morphologic and immunohistochemical features, the neoplasm was eventually defined as malignant peripheral nerve sheath tumor. Histologic examination following radical surgery revealed the neoplasm was confined to the uterine cervix (FIGO stage IB1) and at 12 mo of follow-up, the patient is still free of disease. Malignant peripheral nerve sheath tumors are highly aggressive sarcomas that can rarely involve the uterine cervix. They have to be differentiated from melanoma, leiomyosarcoma, endometrial stromal sarcoma, synovial sarcoma, and other spindle cell neoplasms.


Journal Details

This article was published in the following journal.

Name: International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists
ISSN: 1538-7151


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Medical and Biotech [MESH] Definitions

Neoplasms which arise from nerve sheaths formed by SCHWANN CELLS in the PERIPHERAL NERVOUS SYSTEM or by OLIGODENDROCYTES in the CENTRAL NERVOUS SYSTEM. Malignant peripheral nerve sheath tumors, NEUROFIBROMA, and NEURILEMMOMA are relatively common tumors in this category.

Neoplasms which arise from peripheral nerve tissue. This includes NEUROFIBROMAS; SCHWANNOMAS; GRANULAR CELL TUMORS; and malignant peripheral NERVE SHEATH NEOPLASMS. (From DeVita Jr et al., Cancer: Principles and Practice of Oncology, 5th ed, pp1750-1)

The lipid-rich sheath surrounding AXONS in both the central and peripheral nervous systems. The myelin sheath is an electrical insulator and allows faster and more energetically efficient conduction of impulses. The sheath is formed by the cell membranes of glial cells (SCHWANN CELLS in the peripheral and OLIGODENDROGLIA in the central nervous system). Deterioration of the sheath in DEMYELINATING DISEASES is a serious clinical problem.

A class of nerve fibers as defined by their structure, specifically the nerve sheath arrangement. The AXONS of the unmyelinated nerve fibers are small. The axons to SCHWANN CELLS ratio is greater in the unmyelinated nerve fibers than that in the myelinated fiber (NERVE FIBERS, MYELINATED) which is 1:1. Usually several axons are surrounded by a single Schwann cell in the unmyelinated nerve fibers. Therefore, each unmyelinated fiber is not completely covered by the MYELIN SHEATH formed by the Schwann cell. Unmyelinated nerve fibers conduct impulses at low velocities. They represent the majority of peripheral sensory and autonomic fibers. They are also found in the spinal cord and brain.

Incompetent UTERINE CERVIX is usually diagnosed in the second trimester of PREGNANCY. It is characterized by passive painless cervical dilation in the absence of UTERINE CONTRACTION; BLEEDING; INFECTION; and sometimes with the amniotic sac (AMNIOTIC MEMBRANE) bulging through the partially dilated cervix. Left untreated, this condition may lead to premature pregnancy loss, such as HABITUAL ABORTION.

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