Malignant granular cell tumor of the thigh.
Summary of "Malignant granular cell tumor of the thigh."
Malignant granular cell tumor is a rare neural tumor characterized by abundant granular-appearing tumor cells. These tumors account for <2% of all granular cell tumors. Unlike its benign counterpart, a malignant granular cell tumor presents primarily in the lower limb and is notably larger. Both the uncommon occurrence of malignant granular cell tumors and its similarities in feature with their benign counterparts make diagnosis of this particular malignancy difficult. By 1998, Fanburg-Smith et al developed a diagnostic criteria in which granular cell tumors were divided into 3 categories-benign, atypical, and malignant-based on 6 histological characteristics of the tumor: necrosis, spindling, vesicular nuclei with large nucleoli, increased mitotic activity, high nuclear-to-cytoplasmic ratio, and pleomorphism.This article presents a case of a large malignant granular cell tumor in the right thigh of a 69-year-old woman. Gross examination of the mass showed the well-demarcated, tan, white tumor measuring 18.2 cm long and 7.6 cm wide at its largest width. Histological examination of the mass, performed by an oncological pathologist, demonstrated foci of tumor necrosis, scattered apoptotic cells, prominent nucleoli, increased nuclear-to-cytoplasmic ratio, increased mitotic activity, and areas of spindling with significant atypia.To our knowledge, this is the largest reported case of malignant granular cell tumor in the lower limb diagnosed using the histological criteria established by Fanburg-Smith et al. This case stresses the importance of thorough evaluation in instances of atypical granular cell tumor presentations.
This article was published in the following journal.
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/21815590
- DOI: http://dx.doi.org/10.3928/01477447-20110627-31
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Medical and Biotech [MESH] Definitions
Unusual tumor affecting any site of the body, but most often encountered in the head and neck. Considerable debate has surrounded the histogenesis of this neoplasm; however, it is considered to be a myoblastoma of, usually, a benign nature. It affects women more often than men. When it develops beneath the epidermis or mucous membrane, it can lead to proliferation of the squamous cells and mimic squamous cell carcinoma.
An unusual and aggressive tumor of germ-cell origin that reproduces the extraembryonic structures of the early embryo. It is the most common malignant germ cell tumor found in children. It is characterized by a labyrinthine glandular pattern of flat epithelial cells and rounded papillary processes with a central capillary (Schiller-Duval body). The tumor is rarely bilateral. Before the use of combination chemotherapy, the tumor was almost invariably fatal. (From DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1189)
A bone tumor composed of cellular spindle-cell stroma containing scattered multinucleated giant cells resembling osteoclasts. The tumors range from benign to frankly malignant lesions. The tumor occurs most frequently in an end of a long tubular bone in young adults. (From Dorland, 27th ed; Stedman, 25th ed)
A malignant tumor composed of more than one type of neoplastic tissue. (Dorland, 27th ed)
A group of carcinomas which share a characteristic morphology, often being composed of clusters and trabecular sheets of round "blue cells", granular chromatin, and an attenuated rim of poorly demarcated cytoplasm. Neuroendocrine tumors include carcinoids, small ("oat") cell carcinomas, medullary carcinoma of the thyroid, Merkel cell tumor, cutaneous neuroendocrine carcinoma, pancreatic islet cell tumors, and pheochromocytoma. Neurosecretory granules are found within the tumor cells. (Segen, Dictionary of Modern Medicine, 1992)