Hyalinizing spindle cell tumor with giant rosettes is a rare soft tissue neoplasm. It occurs on the extremities. Its name is descriptive of its microscopic appearance. It appears to show features of both mesenchymal and neuroendocrine differentiation.
Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/Other Diagnostic Testing Gross Appearance and Clinical Variants Histopathological Features and Variants Special Stains/Immunohistochemistry/Electron Microscopy Differential Diagnosis Prognosis Treatment Commonly Used Terms Internet Links
EPIDEMIOLOGY CHARACTERIZATION INCIDENCE Rare AGE RANGE-MEDIAN Middle aged adults
PATHOGENESIS CHARACTERIZATION CHROMOSOMAL ALTERATIONS
Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes share a common t(7;16)(q34;p11) translocation.
Reid R, de Silva MV, Paterson L, Ryan E, Fisher C.
University Department of Pathology/Scottish Bone Tumour Registry Western Infirmary, Glasgow, United Kingdom.
Am J Surg Pathol. 2003 Sep;27(9):1229-36. Abstract quote
Low-grade fibromyxoid sarcoma (LGFMS) is a rare metastasizing soft tissue tumor with deceptively bland histologic features. The hyalinizing spindle cell tumor with giant rosettes (HSCT) is thought to be a closely related tumor differing only by the presence of collagen rosettes.
We report the occurrence of a common t(7;16)(q34;p11) translocation in 2 cases of HSCT and 2 cases of LGFMS, thereby providing the first cytogenetic proof that LGFMS and HSCT are variants of the same entity. The tumors occurred in the thighs of 2 females and in the buttock and supraclavicular fossa of 2 males. One HSCT had a spectrum of unusual histologic features, including the presence of plump epithelioid cells with abundant cytoplasm and strands and nests of clear epithelioid cells separated by eosinophilic hyalinized stroma. Two cases showed a hitherto unreported, focal staining with epithelial membrane antigen, thus adding to the immunohistochemical profile of these tumors. LGFMS and HSCT probably have a wider spectrum of morphologic features than previously thought, the awareness of which will help pathologists to avoid diagnostic pitfalls.
Demonstration of the t(7;16)(q34;p11) translocation will help to diagnose difficult cases with unusual histologic features.
Possible neuroendocrine orgin
LABORATORY/RADIOLOGIC/OTHER TESTS CHARACTERIZATION Cytogenetics Two cell lines containing balanced translocation between chromosomes 7 and 16
GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION Classic Well-circumscribed surrounded by a thin capsule-like fibrous band
HISTOLOGICAL TYPES CHARACTERIZATION Classic
Two architectural components:
Giant rosettes composed of hyalinizined acellular islands surrounded by round to oval plump cells in a palisading-like pattern
Occasionally arranged in a serpiginous connection
Birefringent core with polarized light
Occasional ghost cells in the center
Hypercellular areas with plump elongated cells with highly vascular background
Rare multinucleated cells
No necrosis and rare mitotic figure
Low-Grade Fibromyxoid Sarcoma and Hyalinizing Spindle Cell Tumor With Giant Rosettes A Clinicopathologic Study of 73 Cases Supporting Their Identity and Assessing the Impact of High-Grade Areas
Andrew L. Folpe, M.D.; Kathryn L. Lane, M.D.; Gerson Paull, M.D.; Sharon W. Weiss, M.D.
From the Department of Pathology and Laboratory Medicine, Emory University, Atlanta, Georgia, U.S.A. (A.L.F., G.P., S.WW.); and Huntsville Pathology Associates, Huntsville, Alabama, U.S.A. (K.L.L.).
Am J Surg Pathol 2000;24:1353-1360 Abstract quote
Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma characterized by bland histologic features and a paradoxically aggressive clinical course. The hyalinizing spindle cell tumor with giant rosettes (HSCT) is a closely related tumor characterized by the presence of giant collagen rosettes. Only a single example of a metastasizing HSCT has been reported. A small subset of both LGFMS and HSCT display areas of increased cellularity and atypia which qualify as intermediate-to high-grade sarcoma; the significance of these features has not been definitively assessed.
We present the clinicopathologic features of 77 cases of LGFMS and HSCT to determine the degree of overlap of these two lesions, their biologic behavior, and the significance of the occasional presence of intermediate-to high-grade sarcoma within both.
The patients (33 female, 40 male) ranged from 3 to 78 years of age (median, 34 yrs). Fourteen cases occurred in patients less than 18 years of age. The tumors measured from 1 to 23 cm (median, 4.5 cm) and occurred predominantly in the trunk and lower extremities in both the deep (66 cases) and superficial (7 cases) soft tissues. In 15 cases, the tumor was present >1 year before diagnosis. All tumors showed predominantly the typical hypocellularity and bland cytologic features of typical LGFMS; however, areas of hypercellularity and nuclear enlargement and hyperchromatism were identified in 12 of 73 (16%) and 7 of 73 (10%), respectively. Necrosis and mitotic activity >5/50 high-powered fields (HPF) were present in 6 of 73 (8%) and 5 of 73 (7%), respectively. Epithelioid areas were present in 33 of 73 (45%) and rosettes in 22 of 73 (30%). Follow up (54 cases; range, 2–192 mos; median, 24 mos; mean, 38 mos) showed 5 recurrences, 3 metastases, and 1 death. The diagnosis of LGFMS or HSCT was made prospectively in 51 patients; none had metastatic disease. Two of the metastatic tumors were LGFMS and one was a HSCT. LGFMS may occur more often in the pediatric population and show a much wider histologic spectrum than previously thought. A significant number of LGFMS possess inconspicuous collagen rosettes characteristic of HSCT, indicating that these two tumors are ends of a common spectrum rather than distinct entities.
HSCT, like LGFMS, are low-grade sarcomas with metastatic potential. The presence of focal areas of intermediate-to high-grade sarcoma does not portend a worse outcome in the short term. The better prognosis reflected in this study compared with previous ones might reflect the fact that all were initially diagnosed as sarcomas and treated with aggressive surgery. The fact that the only three patients to develop metastatic disease were patients whose LGFMS or HSCT was identified retrospectively supports this concept.
SPECIAL STAINS/IMMUNOPEROXIDASE/OTHER CHARACTERIZATION Rosettes S-100, CD57, NSE+ Spindle cells HAM56, Factor XIIIa, CD68+
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES Fibrosarcomatous DFSP with giant rosettes
Am J Dermatopathol 2001;23:41-45
Tumor contained areas of low grade fibrosarcoma with giant rosettes, resembling cases of hyalinizing spindle cell tumor with giant rosettes (HSCTGR)
Both had CD34 positive areas suggesting a link between the two tumors
PROGNOSIS AND TREATMENT CHARACTERIZATION 5 Year Survival Metastasis
Am J Surg Pathol 1999;23:1425-1428
Rare cases indicating behavior of a low grade fibrosarcoma
Treatment Complete excision
Am J Surg Pathol 1997;21:1481-1488
Arch Pathol Lab Med 2000;124:1179-1184.
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