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Precise preoperative diagnosis of three malignant nerve sheath tumors (MNST) was based on their remarkably uniform and highly characteristic cytologic appearance. The differential diagnosis with benign nerve sheath tumors and other spindle cell sarcomas is addressed, and the possibility of achieving confident diagnosis on cytologic material from at least some soft-tissue sarcomas is confirmed.
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The development of sarcoma subsequent to cranial irradiation is a rare but serious and potentially fatal event. We describe seven patients who had undergone cranial irradiation (range, 1600-6000 cGy) to treat their primary disease and who developed sarcomas within the irradiated field. The median time from radiation therapy to the development of a sarcoma was 8 years (range, 4 to 15 yr). Fibrosarcomas developed in four patients, and malignant fibrous histiocytomas developed in three. Despite aggressive treatment, the prognosis was poor; the median survival from the diagnosis of sarcoma was 19 months. Sarcoma should be considered in the differential diagnosis of a new lesion or a lesion that progresses several years after radiation therapy.
The clinical courses are presented for 6 patients with sarcoma of the spermatic cord seen between 1967 and 1976. There were 3 fibrosarcomas, 2 liposarcomas and 1 malignant fibrous histiocytoma, making the total reported cases of spermatic cord sarcoma 212. Management of spermatic cord sarcoma is discussed.
Postradiation sarcomas are rare, and the most commonly reported ones are malignant fibrous histiocytoma, osteosarcoma, angiosarcoma, fibrosarcoma, malignant peripheral nerve sheath tumor, and high-grade pleomorphic sarcoma, not otherwise specified. There are a few case reports of postradiation rhabdomyosarcomas following treatment of retinoblastoma, breast cancer, endometrial adenocarcinoma, and Hodgkin's disease. Secondary neoplasms following radiation and surgical treatment of rectal adenocarcinomas have not been reported in the English literature. We report a case of pleomorphic rhabdomyosarcoma of the anterior abdominal wall following treatment of rectal carcinoma, and we review the literature.
Six cases of primary hepatic carcinomas with a significant amount of sarcomatoid elements were examined by using immunohistochemical stainings. Four of the six cases were associated with ordinary hepatocellular carcinoma (HCC), one with cholangiocellular carcinoma (CCC), and one with mixed HCC and CCC. Alpha-fetoprotein and alpha-1-antitrypsin were negative in sarcomatoid cells of all cases; vimentin stained positively in sarcomatoid tumor cells in two of the six cases; and cytokeratin (CK8) was detected in five cases. The CK8 was not detected in tumor cells of two cases of hepatic angiosarcoma, two of metastatic leiomyosarcomas, and one of metastatic fibrosarcoma, although vimentin stained positively in all these true sarcomas. It was concluded that sarcomatoid dedifferentiation of liver carcinomas might derive from both HCC and CCC. In addition CK8 might be an excellent marker to make a differential diagnosis of sarcomatoid cancers from true metastatic or primary sarcomas of the liver.
Histopathologic peer review of specimens from 216 consecutive patients with soft-tissue or bone sarcomas on Southeastern Cancer Study Group protocols was performed by a panel of three pathologists. Subtype of sarcoma, degree of confidence in diagnosis, and grade were compared with agreement or disagreement in pathologic opinion from the primary member institution v the pathology review panel. The most common soft-tissue sarcoma was leiomyosarcoma, followed by malignant fibrous histiocytoma, fibrosarcoma, liposarcoma, malignant schwannoma, and rhabdomyosarcoma. There was total agreement between the primary pathologist and reviewer in 66% of cases. However, 12 cases (6%) were considered on review to not be sarcomas. In 27% of cases the subtype of sarcoma was felt to be incorrect by reviewers. Discordant diagnoses were more common when the confidence of the pathologist was less and when the tumor was of higher grade. There was no improvement in frequency of agreement in the second half of the study, despite educational workshops. We conclude that histologic peer review is important in sarcoma studies and is essential in many patients with presumed sarcomas.
Non phylloides sarcomas of the breast are infrequent malignant tumors that can be identified only at histologic examination since clinical presentation may not differ from the more frequent phylloides type. In addition to fibrosarcoma, leiomyosarcoma, rhabdomyosarcoma, angiosarcoma and liposarcoma, the stromal sarcoma of the breast has been recently identified. We report 3 such cases with heterologous components of osteosarcoma, chondrosarcoma and liposarcoma. A third case with an anti-sarcoma appearing post radiotherapy for breast carcinoma is also reported.
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BACKGROUND: Primary brain sarcomas are rarely curable with surgery and standard radiation therapy. They typically recur locally within 6 months of treatment. This case report describes a novel treatment approach for primary or recurrent brain sarcomas with intracavitary brachytherapy. CASE DESCRIPTION: This 34-year-old female presented with a large and rapidly recurrent primary fibrosarcoma in the right fronto-parietal brain only 1 month postinitial total resection. She was reoperated, again with an MRI-documented gross total resection, but at this second surgery a GliaSite RTS (a recently FDA-approved balloon catheter system for intracranial intracavitary brachytherapy) was inserted into the surgical cavity. Over four days a radiation dose of 152 Gy was delivered at the balloon surface dose and 50.0Gy was delivered at a depth of 7 mm from balloon surface. The patient received subsequent treatment with external beam radiation and chemotherapy. The patient tolerated her treatment well and has shown no evidence of tumor recurrence with a follow up of 18 months. CONCLUSIONS: Boost intracavitary brachytherapy can play a critical role in preventing local recurrence and early death in patients with primary brain sarcomas.
It was reported that angiotensin II stimulates angiogenesis in vivo, and angiotensin-converting enzyme (ACE) inhibitors inhibit angiogenesis. We found that an AT1-receptor (AT1-R) antagonist, TCV-116, inhibited tumor growth, tumor-associated angiogenesis, and metastasis in a murine model. Tumor growth of Sarcoma 180 (S-180) cells and of fibrosarcoma (NFSA) cells was strongly inhibited by administration of TCV-116 in the diet at a dose of approximately 100 mg/kg/day. This reduction was accompanied with a marked reduction in tumor-associated angiogenesis. The same treatment also reduced the lung metastasis of intravenously injected Lewis lung carcinoma cells. These effects of TCV-116 were equivalent to those of the ACE inhibitor, lisinopril. In S-180 and NFSA tumor tissues, ACE and AT1a receptor (AT1a-R) mRNAs were expressed when assessed with RT-PCR. AT1b receptor and AT2 receptor, however, were not detected. Immunoreactive AT1-R was detected mainly on the neovascularized vascular endothelial cells in which expression was reduced by TCV-116 and lisinopril. These results suggested that TCV-116 inhibits the angiogenesis, growth, and metastasis of tumors highly dependent on AT1a-R blockade. Blockade of AT1a-R signaling may therefore become an effective novel strategy for tumor chemoprevention.
Tumor-bearing mice showed a significant resistance against Candida albicans intravenous infection. Longer survival was observed in groups of mice inoculated with fungal cells 2-3 weeks after tumor transplantation with allogeneic sarcoma 180, syngeneic methylcholanthrene-induced Meth A fibrosarcoma, and MM 46 mammary carcinoma than in non-tumor-bearing mice inoculated only with fungal cells. This effect was not observed when the mice were infected only 1 week after tumor transplantation. A significant decrease in the number of C. albicans cells in the kidneys was observed in mice inoculated with fungal cells 2-3 weeks after tumor transplantation. In the tumor-bearing mice treated with cyclophosphamide (CY), a remarkable decrease in both the number of peripheral blood polymorphonuclear leukocytes (PMNs) and the defense against challenge with C. albicans cells was observed, as compared with the CY-untreated groups (normal and tumor-bearing mice). A marked increase in the calcium concentration in serum and number, candidacidal activity, active oxygen level, and myeloperoxidase activity of PMNs was observed in the 2-3-week tumor-bearing mice. From these results, it is suggested that PMNs, which accumulated in the 2-3-week tumor-bearing mice, play an important role in the protection from C. albicans infection by increasing the number and the types of killing factors.
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We report the clinical and pathologic features of 32 sarcomas of the breast with features spanning the spectrum of fibrosarcomas-malignant fibrous histiocytomas. Neoplasms were categorized as high- or low-grade lesions depending on a combination of the degrees of atypia and mitotic activity. The majority of high-grade lesions had marked (3+) nuclear atypia and at least five mitotic figures per 10 hpf. High-grade lesions with moderate (2+) nuclear atypia had a mitotic activity of six or more mitotic figures per 10 hpf. All low-grade lesions had five or fewer mitotic figures per 10 hpf, and none had a score of the nuclear grade times mitotic figures of more than 10. The average mitotic activity in low-grade lesions was two mitotic figures per 10 hpf; the high-grade lesions had 12 mitotic figures per 10 hpf. Sixty-nine percent of the low-grade fibrosarcomas-malignant fibrous histiocytomas showed mild (1+) cytologic atypia, and 69% of the high-grade lesions showed severe (3+) cytologic atypia. The herringbone pattern was associated with a more favorable prognosis than the malignant fibrous histiocytoma pattern. Compared to the high-grade lesions, low-grade fibrosarcomas-malignant fibrous histiocytomas were slow-growing, produced fewer recurrences, and did not metastasize. Of the 16 women with low-grade lesions, all were free of tumor at last contact, despite recurrence in more than half of the patients. In contrast, 31% of the patients with high-grade lesions died of tumor, and 13% were alive with disease. Twenty-five percent of women with high-grade lesions developed distant metastases.
Primary sarcomas of the lung are extremely rare. Among the most common to occur in this location are leiomyosarcoma, fibrosarcoma, and hemangiopericytoma. Many difficulties are encountered when establishing these sarcoma diagnoses, or one of another pathologic type, because of overlapping histologic features and morphologic similarities between primary and metastatic lesions. In this study, the diagnosis of a primary monophasic synovial sarcoma of the lung was aided by the observation of the X;18 translocation characteristic of this neoplasm. To the best of our knowledge, this is the first cytogenetic report of a primary pulmonary synovial sarcoma.
Clear cell sarcomas of tendons and aponeuroses were first described by Enzinger in 1965 and are characterized by the occurrence in young adults, frequent location in the distal lower extremity and slow, but often relentless clinical course. The histologic features of 5 own cases are presented, the cellular arrangement in nest-, cluster- or "ball"-like formations and a prominent nucleolus are emphasized as most reliable diagnostic patterns. Some aspects of differential diagnosis are discussed, especially those concerning its histologic distinction from fibrosarcomas, malignant melanomas, malignant Schwannomas and synovial sarcomas. The electron microscopic examination of one case revealed findings which may be considered as typical for tumors developed from neural crest derived cells: envelopment of many tumor cells by basal lamina-like structures, specialized cellular contacts, interdigitations of cellular processes and "wrapping around"-phenomena (so-called mesocollagen formation). Some neurosecretory granules (not described till now in clear cell sarcomas) and fibrous long spacing collagen further support the suggestion that clear cell sarcomas pertain to the group of tumors with relationship to neuroectodermal cells and tissues. We feel that, because of their typical histologic picture and their particular clinical behavior, they should not be regarded as malignant (soft tissue) melanomas or malignant (melanotic) Schwannomas but retained as a defined clinico-pathological entity.
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