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Sarcoma diagnosis continues to evolve as new information is discovered. Certain tumors have been downgraded (dermal leiomyosarcoma) and an atypical category designed for others. Recently entities include myxoinflammatory fibroblastic sarcoma, myoepithelioma, and pseudomyogenic hemangioendothelioma. The terms malignant fibrous histiocytoma and hemangiopericytoma are outdated. New immunostains (STAT6, SOX10, ERG) add diagnostic specificity, and new risk assessment models are described for sarcomas where grading and staging has failed to provide adequate prognosis., (© 2015 Wiley Periodicals, Inc.)

Spindle-cell hibernoma is 1 of 4 histologic variants of hibernomas, which are rare lipomatous tumors distinguished from other lipomas by their brown fat component. This article presents a case of a spindle-cell hibernoma that developed in the groin of a 58-year-old man, and is 1 of only 5 known cases of spindle-cell hibernoma published in the scientific literature. Minimal information is available regarding the clinicopathologic characteristics of the 4 hibernoma variants: typical, lipoma-like, myxoid, and spindle-cell. Spindle-cell hibernoma is believed to be the rarest variant, accounting for approximately 2% of hibernomas. The spindle-cell variant predominantly develops in the fourth and fifth decades, with an average age at diagnosis of 42.5 years (range, 28-59 years). It has a male predilection of 4:1. Previously reported only in the posterior neck and scalp, the groin is now added to the sites of spindle-cell hibernoma occurrence. Our patient's tumor demonstrated low-attenuation on computed tomography and contained moderately-attenuating internal septae. Histologically, in addition to the multivacuolated brown fat component common to all hibernomas, spindle-cell hibernoma has a spindle-cell element without vacuolization that is CD34 positive. No prior treatment details are available on this particular variant. Our patient was treated by marginal excision and was disease free through 13 months of follow-up., (Copyright 2010, SLACK Incorporated.)

Although cardiac sarcomas are rare in comparison to their soft tissue counterparts, they are the second most common type of primary cardiac neoplasm. Of the few hundred cases reported, most has been based on autopsy series. A series of 27 cardiac sarcomas removed at surgery for curative and diagnostic intent were reviewed for clinicopathologic features with correlation to available postoperative follow-up data in 17 patients. There were 6 angiosarcomas, 6 myxofibrosarcomas, 3 malignant peripheral nerve sheath tumors, 3 leiomyosarcomas, 2 synovial sarcomas, 1 epithelioid hemangioendothelioma, 1 chondrosarcoma, 1 osteosarcoma, and 4 poorly differentiated sarcomas. There was a wide age and size range with slight female predilection. There were 20 cases that arose in the atria/pulmonary vessels, 4 in the ventricles, 1 in mitral valve, and 2 in epi/pericardium. There was a slight left predilection. The histologic grade was low in 4, moderate in 3, and high in 20 cases. Six high-grade and 1 low-grade tumors were also treated with adjuvant chemotherapy and/or radiation. In 17 patients with follow-up data, 6 of 12 patients with high-grade tumor died (4 within 5 days of the initial surgery, 1 in 21 months, and 1 in 131 months), and 1 patient with moderate-grade tumor and all 4 patients with low-grade tumor were alive without evidence of disease at the end of follow-up. Tumor grade appeared to be prognostically important in cardiac sarcoma. Long survival was achieved in patients who survived the initial surgery well.

Metastatic sarcomas are commonly resistant to chemotherapy. The serine/threonine kinase, mammalian target of rapamycin (mTOR), is a protein kinase of the phosphatidylinositol 3-kinase (PI3K)/AKT signaling pathway thought to have a key role in controlling cancer growth and thus is an important target for cancer therapy. Several inhibitors of mTOR are in clinical trials, including AP23573, which is being tested on metastatic sarcomas and other tumors. We hypothesized that a marker for the activity of mTOR, phosphorylated S6 ribosomal protein, would be predictive of clinical response to the drug, that is, high tumor expression would signify better response than low expression. This was a blinded study. Of 26 patients treated, 20 remained on study, with available paraffin blocks. Fourteen patients received AP23573 alone and six patients received AP23573 in combination with adriamycin. An antibody to the phosphorylated S6 ribosomal protein was used to stain the tumors, all high-grade sarcomas. Pretreatment biopsy or resection material was tested: the original tumor (n=6) or tumor recurrence/metastasis (n=14); either of these may have been after treatment with other agents. Staining was scored for both quantity/percentage of tumor cells and intensity. Scoring was performed without knowledge of tumor response. Staining quantity could be categorized into two natural groups: high expressors (> or =20% of tumor cells, 11 cases) and low expressors (0-10% of tumor cells, 9 cases). The high-expression group had eight stable and three progressive cases (73% stable disease); the low-expression group had three stable and six progressive cases (67% progressive disease). Chi-square analysis showed statistical significance (P< or =0.05) at this initial cutoff (10%) selected blindly. The level of phosphorylated S6 ribosomal protein expression was predictive of early tumor response to the mTOR inhibitor, suggesting that this is a promising new predictive sarcoma marker for targeted mTOR inhibitor therapy.

The vast majority of soft tissue tumors (STT), especially sarcomas, should be examined according to a national standard for pathologic evaluation, including a national consensus on a grading system and elements of a synoptic pathology report. The margins should be separated into fascial margins and other surgically cut margins. If questions about the diagnosis arise, a second opinion should be sought from an expert in sarcoma pathology. Whenever possible, tissue should be frozen for potential molecular diagnosis and additional tissue banked for research. Finally, there is a need for a national registry for rare sarcoma types.

The article presents a case report for a 53 year old man with a history of melanoma who presented to orthopedic surgeons with complaints of pain in his right forearm. A discussion of medical tests which were performed on the patient and resulted in him receiving a diagnosis of osteosarcoma of the proximal part of the radius and Mazabraud syndrome is presented. The patient's medical care and recovery are discussed.

Primary sarcomas of the salivary gland region occur rarely and may be confused with other primary salivary gland neoplasms. This distinction is critical, as the treatment of salivary gland sarcomas differs significantly from the therapy of the more common tumors typically found in these glands. We report an unusual case of synovial sarcoma arising within the parotid gland of a 51-year-old female. Large areas were monophasic and resembled hemangiopericytoma, but a focal unequivocal biphasic area was noted. Previous cases reported in this region are reviewed. [ABSTRACT FROM AUTHOR]

Most low-grade endometrial stromal sarcomas are intrauterine in origin, and a primary extrauterine location in the mesentery or omentum is extraordinarily rare. Hence, we report a well-documented, low-grade endometrial stromal sarcoma of extrauterine origin as an incidental finding in a patient who had extensive ovarian intra-abdominal carcinomatosis. This case adds to the literature experience in this entity and illustrates that such lesions may occur as incidental and unexpected findings in patients with other gynecologic neoplasms. [ABSTRACT FROM AUTHOR]

Background Alveolar soft part sarcoma (ASPS) is a rare, malignant soft-tissue neoplasm typically seen in young adults that possesses an unusual tendency to metastasize. Metastases to the intramedullary compartment of the spinal cord, however, are exceptionally rare and have not been described in the literature. Case Description We report the case of a 23-year-old woman with disseminated ASPS to the lung and brain who presented with progressive lower-extremity weakness and loss of sensation after radiation and chemotherapy. Magnetic resonance imaging revealed a 1.3-cm avidly enhancing lesion within the central thoracic spinal cord at T3. A T2–T4 laminectomy was undertaken and resulted in a gross total resection. Histopathologically, the mass was composed of organoid nests containing epithelioid cells with eosinophilic, granular cytoplasm separated by sinusoidal spaces. Immunohistochemistry demonstrated convincing positive TFE3 staining. Postoperative imaging confirmed the complete resection of the mass, and her examination was notable for intact sensation and impaired motor function that gradually improved. Conclusions A review of the literature found that the reported case represents the first instance of primary or metastatic ASPS in the spinal cord. Metastatic ASPS should thus be included in the differential diagnosis in patients with known disease and neurologic impairment or back pain. Imaging of the spine should then be considered. [ABSTRACT FROM AUTHOR]