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Spermatic cord sarcoma is often misdiagnosed by the urologists due to its rarity. One patient with fibroliposarcoma of the spermatic cord and brief review on the diagnosis, critical analysis of the biologic behavior and the therapeutic approach are presented. The authors suggest that the regimen for this lesion be made upon the tumor cell type and the pathologic grade after radical orchiectomy.
Liver involvement by soft tissue sarcoma is an unfavorable prognostic factor for survival. Complete resection of liver metastases can bring improvement in selected patients, but chemotherapy remains the only palliative treatment option for most. Anecdotal long-term survival of patients with unresectable liver metastases treated with systemic chemotherapy has been reported, such as the patient presented here.
A case report is presented of Richter hernia of the stomach, after en bloc excision of multiple organs for sarcoma of the left upper quadrant of the abdomen. To our knowledge this is the first case reported in the literature. The conditions for the development of this hernia are : (1) the freeing of the greater curvature of the stomach (following removal of the spleen and tail of the pancreas); and (2) fascial dehiscence following a left thoracoabdominal incision involving rib resection.
Soft tissue tumours represent a heterogeneous group of mesenchymal lesions, and their classification is the subject of continuous debate. Chromosome analysis, molecular cytogenetics and molecular assays may become increasingly useful in diagnosis, and this review summarises advances in the cytogenetic characterisation and classification of soft tissue tumours. Among the group of fibrous lesions, superficial fibromatosis exhibits trisomy 8. This genomic change is also observed in desmoid fibromatosis in association with trisomy 20. Trisomy 11 is the most frequently observed chromosomal aberration in congenital fibrosarcoma. Dermatofibrosarcoma protuberans and giant cell fibroblastoma share a translocation t(17;22), which supports the concept of the existence of a common differentiation pathway. Adipose tissue tumours is the group in which integration of genetics and pathology has been most fruitful. Ordinary lipomas cytogenetically show an abnormal karyotype in about half the cases. Genomic changes of the 11q13 region are observed in hibernoma. Lipoblastoma exhibits a specific 8q rearrangement in 8q11-q13. Loss of material from the region 16q13-qter and 13q deletions are observed in spindle cell/pleomorphic lipomas. The well-differentiated liposarcoma/atypical lipoma group is characterised karyotypically by the presence of one extra ring and/or extra giant chromosome marker. Myxoid and round cell liposarcoma share the same characteristic chromosome change: t(12;16)(q13;p11) in most cases. In the group of smooth muscle lesions most data are derived from uterine leiomyomas, which can be subclassified cytogenetically into seven different types. Half of all leiomyomas are chromosomally normal; the other half have one of six possible consistent chromosome changes. Alveolar rhabdomyosarcoma is characterised cytogenetically by two variant translocations t(2;13)(q35;q14) and t(1;13)(p36;q14). Among tenosynovial tumours, the localised type of giant cell tumour of tendon sheath exhibits two different karyotypic changes. One involves 1p11 in a translocation with chromosome 2 or with another chromosome. A second type involves 16q24. Synovial sarcoma is characterised cytogenetically by a translocation occurring between chromosome 18 and presumably two adjacent loci on the X chromosome. In neural tumours, abnormalities of chromosome 22 have been reported in benign schwannomas and perineuriomas. Malignant peripheral nerve sheath tumours exist in two main forms: sporadic and associated with the NF-1 syndrome. Karyotypes are very complex, but chromosomes 17q and 22q are very often involved. Clear cell sarcoma is characterised cytogenetically and molecularly by a translocation t(12;22)(q13;q12). The Ewing's sarcoma/peripheral neuroectodermal tumour category shows a central karyotypic anomaly represented by the translocation t(11;22). The two variants t(21;22) and t(7;22) are found in some cases. Among cartilaginous lesion, the most frequently described anomaly is the t(9;22)(q22;q12) in extraskeletal myxoid chondrosarcoma. Intra-abdominal desmoplastic small round cell tumour is characterised by a t(11;22)(p13;q12).
Sarcomas of the spermatic cord are rare, with only approximately 200 such tumors reported in the literature. Of those cases only 3 fit the definition of malignant mesenchymoma: a mesenchymal tumor with 2 or more malignant elements other than fibrosarcoma. We report the fourth such case treated by local excision alone. The patient was free of disease 6 years after treatment.
Pleomorphic carcinoma of the pancreas is a well defined histopathological entity characterized by non-cohesive, sarcoma-like growth pattern, and bizarre mono- and multinucleated tumor giant cells with abundant eosinophilic cytoplasm. Fifteen cases are identified in autopsy files of the Department of Pathology, Washington University School of Medicine, which represent 7.1% of all the non-endocrine pancreatic malignancies found at autopsy. Pleomorphic carcinoma is comparable to pancreatic adenocarcinoma in clinical features such as age, sex, and presenting symptoms except that it is more likely to occur in the body and tail of the pancreas, metastases invariably develop, hematogenous spread is more common, and the median survival is worse. Pleomorphic carcinoma could be distinguished from the pancreatic tumors that resemble giant cell tumor of the bone. Differential diagnostic features between it and amelanotic melanoma, hepatocellular carcinoma, choriocarcinoma, pleomorphic liposarcoma, pleomorphic rhabdomyosarcoma, fibroxanthosarcoma, poorly differentiated epidermoid carcinoma, and giant cell carcinomas of the lung and thyroid are discussed.
OBJECTIVE: Dermatofibrosarcoma protuberans (DFSP) is a relatively rare, slow-growing, fibrohistiocytic sarcoma of intermediate malignancy. The tumor appears most frequently on the trunk and extremities as a slow-growing indurated plaque or nodule. It appears mostly between the second and fifth decades of life. METHODS: This is a case presentation of a 59-year-old woman with a 10 x 12 cm dermal erythematous plaque on the anterior chest wall. RESULTS: The patient has a two year history of painless tumor on the chest. The lesion started as a small erythematous plaque and gradually increased in size. At that time the histologic examination revealed a benign fibrous histiocytoma. Less than two years later the lesion was firm, painless to palpation and movable over deeper structures. The second histologic examination revealed a myxoid liposarcoma. The third incisional biopsy was diagnostic for DFSP showing a dermal tumor consiting of a storiform pattern with cartwheel-like cells arranged around collagenous hubs. The patient underwent a standard surgical treatment. CONCLUSION: We report an interesting case of dermatofibrosarcoma protuberans where limitations of standard vertical histology section resulted in postponed conformation of the diagnosis.
Primary retroperitoneal neoplasms account for only 0.1-0.2% of all malignancies. Retroperitoneal neoplasms are most commonly mesodermal, neurogenic or lymphatic in origin, with lymphoma, liposarcoma, leiomyosarcoma and malignant fibrous histiocytoma the vast majority of malignant primary retroperitoneal tumors. Although the radiographic features of retroperitoneal neoplasms often overlap, certain CT characteristics, pattern of spread and specific tumor prevalence among different demographic groups can help suggest the tumor type. CT is used to diagnosis and assess the size and extent of retroperitoneal tumors, as well as assess the involvement of organs and vasculature with resection in mind.
Five soft tissue sarcomas with histological features of malignant fibrous histiocytoma were selected to illustrate their ultrastructural heterogeneity. One case displayed the mixture of fibroblastic and histiocytic cells characteristic of the majority of malignant fibrous histiocytomas. In 1 case the tumor was composed entirely of primitive mesenchymal cells. The other 3 cases showed lipogenic, neurogenic, and "granular-cell" differentiation, respectively. These findings emphasize the important role of electron microscopy in the precise diagnosis and classification of malignant fibrous histiocytoma.
In a patient presenting with a soft tissue tumor, radiographs are needed to visualize or exclude osseous involvement. Ultrasound can be used to visualize the lesion for cytologic biopsy. If cytology does not rule out sarcoma, magnetic resonance imaging is indicated to stage the lesion. As a rule staging studies other than MR imaging are not needed to evaluate the local situation. Usually MR imaging does not assist in suggesting a specific diagnosis. In certain instances, however, morphology or the presence of high signal intensity on T1, or low signal intensity on T2-weighted images increases specificity. Thick needle biopsy or open biopsy should be performed following MR imaging. This reduces sample errors and increases the accuracy of preoperative staging.
Surgical treatment was performed in 19 patients aged 30 to 64 years with rare giant intrathoracic tumors. The tumor located in the mediastinum in 8 patients, in the lungs -- in 6, in the chest wall with intrathoracic growth -- in 5 patients. Diagnostic examinations included x-ray (including CT) and morphological methods. Sarcoma was diagnosed in 10 patients, mesenchymal tumors -- in 4, neurogenic tumors -- in 3, thymoma -- in 2 patients. Radical surgeries were performed in 15 patients, palliative -- in 3, explorative mini-thoracotomy -- in 1. Computed tomography and CT-assisted puncture biopsy improve the quality of preoperative diagnosis. Radical surgery is the method of choice in surgical treatment of giant benign intrathoracic tumors. Treatment policy is determined individually on the basis of the results of CT, functional and intraoperative findings.
Primary sarcomas of the pleura are rare intrathoracic tumours. We describe a case of a recurring myxoid pleural sarcoma in a 51-year-old male patient with coronary heart disease, who initially was admitted for coronary revascularization surgery. Tumour diagnosis was based on pathological examination of a biopsy after radiological detection of a nodule in the right upper lobe. The nodule was excised. The first recurrence was diagnosed after 15 months. Pleuropneumonectomy was done. Four years after the first excision further tumour masses appeared above the diaphragm and were surgically removed and adjuvant irradiation carried out. A year later the patient is doing well.
We report, for the first time, the cytogenetic and molecular genetic constitution of a human mesenchymoma. As in several other soft tissue sarcomas, supernumerary ring and rod-shaped marker chromosomes were observed next to an otherwise normal diploid karyotype. Comparative genomic in situ hybridization and whole chromosome painting experiments revealed that chromosome 1q21-q25 and 12q14-q15 sequences were amplified, and that these sequences resided on the supernumerary marker chromosomes. We assume that, in this malignant mesenchymoma, the observed chromosomal anomalies may be associated with its well differentiated liposarcomatous component.
An analysis of 50 primary retroperitoneal tumours is presented with emphasis on the urological aspect. 70-80% of the tumours were malignant and sarcomas predominate. The tumour mass was palpable in over 70% of the cases. Frequent involvement of the urinary tract caused lumbar pain in 78% of cases. Intravenous urography and retrograde pyelography are the greatest single aids in accurate diagnosis of primary retroperitoneal tumours since over 70% show abnormalities. Selective angiograms may define the size and operability of the tumour. When diagnosed, extirpation of the tumour is possible in only 30-40% of cases. Postoperative mortality is about 10%. Since 70% of these tumours are radiosensitive, radiotherapy improves the prognosis. Survival for more than 2 years after surgery is exceptional.
Ten patients with sarcoma of the spermatic cord were treated at our hospital between 1940 and 1977. Although there are 191 reported cases in the literature controversy remains concerning optimal treatment. In our series of 10 patients 5 of 7 (71 per cent) followed for more than 5 years postoperatively have suffered local recurrence. Thus, we believe that this treatment is inadequate and recommend postoperative radiation therapy to the scrotum and pelvis. Of our patients 2 suffered recurrence in the retroperitoneal lymphatics and we favor dissection of these nodes as part of the initial treatment.
BACKGROUND: Primary malignant mesenchymoma of the bone is a rare neoplasm consisting of two or more unrelated malignant mesenchymal components. The literature reports fewer than 20 cases, most of which were composed of osteosarcoma and liposarcoma. OBSERVATION: We report an exceedingly rare case of primary malignant mesenchymoma of bone composed of rhabdomyosarcoma, osteosarcoma, and a minor chondrosarcoma component, arising in the right proximal humerus of a 15-year-old girl. The rhabdomyosarcomatous component was present in the initial biopsy and persisted in surgical specimen following chemotherapy. CONCLUSION: Effect of chemotherapy is enigmatic since rhabdomyosarcomatous component could appear, persist or disappear after chemotherapy according to literature.
Non-germ-cell or teratomatous malignant tumors showing additional rhabdomyoblastic differentiation can be divided into three groups. Group I consists of tumors with only sarcomatous differentiation. Included in this group are malignant mesenchymoma, dedifferentiated chondrosarcoma, and the dedifferentiated retroperitoneal liposarcoma. Epithelial or Sertoli-Leydig cell elements represent the second component of tumors in group II. The best known tumor in this group is the malignant mullerian mixed tumor, but other well described tumors with rhabdomyosarcomatous differentiation are the carcinosarcoma, mullerian adenosarcoma, Sertoli-Leydig cell tumor, mammary cystosarcoma, and blastomas. Most of the tumors in groups I and II are derived from mesenchymal tissue. A neuroectodermal origin is shared by all tumors in group III. This includes the medulloblastoma, retinoblastoma and, most frequently, the malignant Triton tumor. The tumors in all three groups are clinically malignant to a varying degree.
Histologically it is often difficult to differentiate malignant fibrous histiocytoma (MFH) from leiomyosarcoma. Sixty-three cases formerly diagnosed as pleomorphic soft tissue sarcomas were examined by immunohistochemistry. We identified three different types according to its positivity for the myogenic markers: A) positive cells with a fascicular pattern, 13 cases (26%); B) a small number of scattered positive cells, 29 cases (57%); C) negative for markers, 9 cases (17%). We reassessed 51 MFHs as follows: A type as pleomorphic leiomyosarcoma, B+C types as MFH. Pleomorphic leiomyosarcomas were also positive for collagen type IV around the compactly arranged individual tumor cells. An imunohistochemical analysis and detection of fascicular structures are thus considered to be necessary in order to distinguish between leiomyosarcoma and MFH.