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Thursday, September 09 2010 @ 11:58 PM CDT

Soft tissues
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Fibrosing fibrosarcomas is a group of predominantly fibrosed or sclerosed tumors primarily affecting the extremities of adults of either sex. Recognition of this group started with the description by Evans of low-grade fibromyxoid sarcoma, a deceptively bland fibrous tumor simulating desmoid tumor, but which had focal myxoid changes and surprisingly a definite metastatic potential. Identification of this tumor has been also hampered by its histologic resemblance to myxofibrosarcoma. More recently, Folpe et al, in a large study encompassing 73 cases, showed that up to 30% of low-grade fibromyxoid sarcomas show overlapping histologic features with hyalinizing fibrosarcoma with giant rosettes. Furthermore, a very recent cytogenetic report demonstrated an identical recurrent t(7;16)(q34;p11) translocation in both of these subtypes of fibrosarcoma, thereby providing genetic proof that these two tumors are variants of the same entity. Ultrastructurally, the unifying concept of fibrosing fibrosarcomas is based on well-developed fibroblastic features of the neoplastic cells embedded in a dense collagenous extracellular stroma and admixed with a peculiar amorphous granular material. Nielsen et al reported the ultrastructural findings of three cases of hyalinizing fibrosarcomas with giant rosettes. The neoplastic cells showed fibroblastic features with long branching RER complexes. In all tumors in addition to a abundant extracellular collagen fibers, large aggregated of granular amorphous material resembling "basement membrane-like" substance was noted admixed with the collagen fibers but also seen within the dilated cisternae.
Hyalinizing Fibromyxoid sarcoma with giant rosettes
Friday, November 13 2009 @ 08:13 AM CST
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The cells of a synovial sarcoma have a microscopic appearance similar to that of the synovium, giving the disease its name, but the actual cells from which the tumour develops are unknown and not necessarily synovial.Synovial sarcoma occurs most commonly in the young, representing about 8% of all soft tissue sarcomas, but about 15-20% of cases in adolescents and young adults.The peak of incidence is before the 30th birthday and males are affected more often than females (ratio around 1.2:1). Two cell types can be seen microscopically in synovial sarcoma. One fibrous type, known as a spindle or sarcomatous cell, is relatively small and uniform and found in sheets. The other is epithelial in appearance. Classical synovial sarcoma has a biphasic appearance with both types present. Synovial sarcoma can also appear to be poorly differentiated or to be monophasic fibrous, consisting only of sheets of spindle cells.Most, and perhaps all, cases of synovial sarcoma are associated with a reciprocal translocation t(x;18)(p11.2;q11.2). There is some debate about whether the molecular observation itself is definitional of synovial sarcoma.The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18).
Synovial Sarcoma
Tuesday, July 21 2009 @ 11:42 AM CDT
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Predominantly subcutaneous lesion composed of mature fat and bland spindle cells. - must be located in posterior neck, upper back and shoulders - If located in other sites, must be restricted to dermis or submucosa - Variable amount of adult fat , no lipoblasts - Composed of small bland spindle cells with scant cytoplasm, elongated nuclei and no mitotic figures - Bundles of dense ropy collagen - Stroma may be myxoid - CD34 positive, usually extensive - Frequent histologic overlap with pleomorphic lipoma
Spindle cell lipoma
Tuesday, July 21 2009 @ 11:42 AM CDT
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Location of lesion, rapidity of growth, and self-limited nature same as those of nodular fasciitis The tumor is benign and composed of large basophilic cells resembling ganglion cells.
Proliferative faciitis
Tuesday, July 21 2009 @ 11:42 AM CDT
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Microscopically, the tumour is lobulated and an incomplete shell of mature bone is noted in the area corresponding to the fibrous septa. The tumour consists of lobules of uniform rounded cells arranged in cords or strands. These cells are set in a fibromyxoid stroma. Mitotic figures are inconspicuous.
Ossifying fibromyxoid tumour of soft parts
Tuesday, July 21 2009 @ 11:42 AM CDT
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Nodular fasciitis, a reactive myofibroblastic lesion like proliferative fasciitis, is one of the most common soft tissue lesions encountered by the general surgical pathologist. It is reactive and many cases are associated with a history of recent trauma. The typical clinical history is that of a rapidly growing mass, 2 to 3 centimeters, in the forearm of a young adult. It has a broad morphologic spectrum. Nodular fasciitis is generally circumscribed; it involves the subcutis, fascia or skeletal muscle. The classic example of nodular fasciitis consists of fibroblasts and myofibroblasts arranged in a haphazard to loose storiform pattern, with a variable degree of myxoid change in the stroma, extravasated erythrocytes, and scattered inflammatory cells. Mucoid cysts, keloid-like strands of collagen, intravascular growth (Intravascular fasciitis) and multinucleated giant cells may also be seen. Mitoses (almost never atypical) are usually frequent and should not be equated with malignancy. The nuclei of the proliferating fibroblasts are cytologically bland; the chromatin is fine, nuclear contours smooth, and nucleoli small and basophilic. The cytoplasm ranges from slightly basophilic to eosinophilic, depending on the myofibroblastic component and amount of collagenization. Nodular fasciitis is benign and generally self-limiting, although occasional cases may recur if incompletely excised.
Nodular faciitis
Friday, July 17 2009 @ 11:15 AM CDT
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Most intramuscular hemangiomas occur in young adults, with 80 to 90% presenting before the age of 30. Both sexes are equally affected and most occur within the thigh. There is usually no discoloration of the overlying skin because of the deep location of the tumors. Arteriography is frequently diagnostic. Intramuscular hemangiomas may be predominantly capillary, cavernous, or an admixture of both types. The capillary type is the most common type and may be mistaken as a malignant vascular neoplasm. Small capillaries with plump nuclei are insinuated between adipocytes and skeletal muscle fibers, frequently causing atrophy of the latter. The cavernous consists of large caliber blood vessels, primarily veins, sometimes with pleboliths, which can be detected radiographically. Frequently there is a prominent adipose tissue component, presumable related to muscular atrophy. Such lesions were referred to as benign mesenchymomas in the past. While benign, recurrence of intramuscular hemangiomas is not uncommon due to incomplete excision, which is in turn attributable to its infiltrative growth pattern.
Intramuscular hemangioma
Friday, July 17 2009 @ 11:15 AM CDT
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Extra-abdominal desmoids are deep seated fibroblastic and myofibroblastic proliferations that in essence recapitulate the appearance of a hypercellular scar. All of the fibromatoses (which are a group of biologically disparate lesions that includes the superficial and deep fibromatoses) are characterized by sweeping fascicles of elongated, slender fibroblasts separated by varying amounts of collagen and a myxoid matrix. The cellularity is variable, but never approaches that of a fibrosarcoma, a tumor with which it is frequently confused. Nuclei are cytologically bland, and mitoses may be numerous; the latter have no bearing on prognosis. Parallel-running thin walled, elongate vascular channels are a hallmark of desmoid. Desmoids are insidiously infiltrative lesions, frequently infiltrating skeletal muscle and fat. The degree of infiltration is frequently not appreciated at the time of surgery or frozen section, and in fact may not be apparent grossly. Deep desmoids (fibromatosis) may be abdominal (along the abdominal wall, usually in pregnant women), intra-abdominal (13% associated with Gardner's syndrome) or extra-abdominal (trunk, extremities). The latter type is what is most commonly seen in the Soft Tissue Pathology Department. The treatment of choice is complete excision, which may not be possible or feasible, depending on the anatomic location of the lesion. The initial recurrence rate ranges between 40 to 60% and increases with each recurrence. Despite their locally aggressive and destructive behavior, desmoids do not metastasize. However, they are capable of causing substantial morbidity and even death, particularly within the abdominal cavity (where they may cause obstruction of a viscus or other vital organs) or in the head and neck region. Multicentricity should not be confused with metastases.
Extrabdominal desmoid tumor
Friday, July 17 2009 @ 11:15 AM CDT
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Dedifferentiated liposarcoma is defined as well-differentiated liposarcoma adjacent to an alipogenic (usually MFH-like) spindled tumor. Dedifferentiation may occur de novo or in a recurrence of a well differentiated liposarcoma. The overall incidence of the latter is approximately 10% with the majority representing retroperitoneal tumors. Roughly 20% of dedifferentiated liposarcomas metastasize, but others may revert to pure well differentiated liposarcoma in recurrences, rendering the clinical progression rather unpredictable. The finding of supernumerary ring and giant marker chromosomes in atypical lipomatous tumors in all sites speaks for a common histogenesis. Many observers now agree that the occurrence of dedifferentiation is related to the size and clinical duration of the tumor. Hence it is most prevalent in the retroperitoneum where detection is likely to be delayed, and complete resection more difficult, and vanishingly rare in the subcutis for the opposite reasons. Sarcomas infiltrating non-neoplastic fat may be confused with dedifferentiated liposarcoma, but the latter is identified by the presence of lipoblasts and/or atypical adipocyte nuclei in the fatty component.
Dediferentiated liposarcoma
Friday, July 17 2009 @ 11:15 AM CDT
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Malignant mesenchymal tumor of adults, with a highly pleomorphic morphology, exhibiting some differentiation into skeletal muscle, without foci of alveolar or embryonal type of rhabdomyosarcoma. Immunohistochemical Stains: Myogenin, myoD1, Desmin, Myosin, Myoglobin
Pleomorphic rhabdomyosarcoma
Friday, May 15 2009 @ 02:26 PM CDT
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Angiolymphoid hyperplasia with eosinophilia also known as epithelioid hemangioma is a distinctive vascular lesion which usually occurs during early and mid adult life (20- 40 years).Microscopically, these are well circumscribed lesions usually located in the dermis and subcutis. Angiolymphoid hyperplasia with eosinophilia consists of vascular and inflammatory cellular components.The vascular component displays thick and thin walled vessels lined by plump endothelial cells. The epithelioid endothelial cells have rounded nuclei abundant eosinophilic cytoplasm containing occasional vacuoles that represent primitive vascular lumen formation. These endothelial cell protrude into the vessels in a 'tombstone' pattern. Within some thicker vessels there is intravascular proliferations of the endothelial cells. Differential diagnosis Kimura disease epithelioid hemangioendothelioma epithelioid angiosarcoma Immunohistochemistry - CD31 + - factor VIIIrAg + - CD34 + - +/- keratins - +/- focal muscle-specific actin-positive myopericytic cells
Epithelioid Hemangioma
Saturday, August 02 2008 @ 04:38 PM CDT
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Desmoplastic small round cell tumor (DSRCT) is a highly malignant neoplasm that was first described in 1989 by Gerald and Rosai. It usually affects young persons during the second decade of life with a striking male predilection. Pathology reveals well circumscribed solid tumor nodules within a dense desmoplastic stroma. Often areas of central necrosis are present. Tumor cells have hyperchromatic nuclei with increased nuclear/cytoplasmic ratio. On immunohistochemistry, these cells have trilinear coexpression including the epithelial marker cytokeratin, the mesenchymal markers desmin and vimentin, and the neuronal marker neuron-specific enolase. Thus, although initially thought to be of mesothelial origin due to sites of presentation, it is now hypothesized to arise from a progenitor cell with multiphenotypic differentiation.
Desmoplastic Small Round Blue Cell Tumor
Saturday, August 02 2008 @ 04:38 PM CDT
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