Case report 100. Poorly differentiated plasma cell myeloma with widespread involvement of soft tissues and multiple organs.
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Mesenchymal chondrosarcoma is an uncommon small-cell neoplasm of bone and soft tissue, the chondrogenic nature of which has been generally accepted. However, the phenotypic attributes of the small-cell population in this neoplasm have not been well characterized, and its relationship to "precartilage mesenchyme" remains unclear. In an attempt to address this issue, we performed an immunohistochemical analysis of nine cases, using antibodies to vimentin, S100 protein, Leu-7 antigen, neuron-specific enolase, synaptophysin, desmin, muscle-specific actin, cytokeratin, and epithelial membrane antigen, and the avidin-biotin-peroxidase complex (ABC) method. The small cells of mesenchymal chondrosarcoma failed to express S100 protein, whereas all components of the tumors (small cells, lacunar chondroblasts, and chondroid matrix) stained for Leu-7 antigen in six cases. Neuron-specific enolase was identified in the small cells of four cases and in the lacunar cells of seven. None contained desmin, actin, cytokeratin, epithelial membrane antigen, or synaptophysin. The immunophenotype of mesenchymal chondrosarcoma resembled that of embryonic cartilage and thus did not contradict the premise that this tumor was the neoplastic counterpart of fetal chondroid tissues. However, immunohistologic studies are not overly helpful in the differential diagnosis between mesenchymal chondrosarcoma and other small round cell lesions.
PURPOSE: To analyze the effectiveness of core-needle biopsy for evaluation of possible primary musculoskeletal neoplasms, which often are evaluated with open biopsy. MATERIALS AND METHODS: Core-needle biopsy was performed at a tertiary care institution in 141 patients suspected of having a mesenchymal neoplasm. In 85 patients, the lesion was in soft tissue; in 56 patients, the lesion was in bone. Eighty-nine patients had a malignant lesion, and 52 had a benign lesion. Twenty-eight patients had undergone previous surgery. RESULTS: In 105 (74%) patients, core-needle biopsy results were concordant with results from specimens subsequently obtained at surgery with respect to tumor histologic features and grade, or they provided sufficient diagnostic information to obviate surgery. In 36 (26%) patients, inaccurate core-needle biopsy results were obtained: In nine, results were imprecise about exact histologic features; in three, results were correct about histologic features but incorrect about tumor grade. In 25 (18%) patients, open biopsy was performed after core-needle biopsy. The accuracy and rate of performance of open biopsy for soft-tissue lesions were not significantly different from those for bone lesions. CONCLUSION: Percutaneous core-needle biopsy can be an effective alternative to open biopsy in the evaluation of possible mesenchymal neoplasms of either bone or soft tissue. Needle biopsy of such lesions, however, is best performed as part of a multidisciplinary team approach to tumor management.
In 1999, we began a study to assess the long-term effect of connective tissue cancer treatment on clinical, social, and psychologic aspects of the lives of surviving patients. A specially designed computer program generated an 85-item questionnaire, which was sent to more than 2000 patients with malignant bone and soft tissue neoplasms. Twelve hundred forty-four patients responded. The data were entered into a computer system and were correlated with the clinical information already contained in the system for the individual patients. Although there are many possible uses for these data, we chose to do a study comparing the lifestyle and physical and sociologic problems for 144 patients treated with chemotherapy and surgery for high-grade osteosarcoma against a control population consisting of 61 patients treated surgically for benign giant cell tumors of bone. The data show a remarkable degree of compensation on the part of the patients with the malignant tumors in terms of some problems but some significant differences particularly in physical status and functional limitations.
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Since first reported by Montgomery in 1927, ossifying fibroma, a benign neoplasm of bone or bone tissue, has been the subject of numerous studies. In the present paper, a case of ossifying fibroma in a woman is presented. The woman was 49 years old. She visited our clinic with a complaint of swelling in the right maxillary region. Thorough examinations were conducted. From X-ray, CT, scintigraphy and biopsy findings, the case was diagnosed as ossifying fibroma. Under a general anesthetic, the tumor was extirpated. It measured 40 x 40 x 60mm. It's weight, including that of teeth, was 60.5 g. Histopathological examination revealed fibroblasts and hard, bone-like masses of varying shapes in the connective tissue. Nearly one year has elapsed since the surgical operation. The patient is doing well. Since 1957, a total of 107 cases of ossifying fibroma have been reported in Japan. The ages of the patients were mostly between 10 and 49. By sex, 42 were male and 65 female. By region, in 28 case, fibroma occurred in the maxillary part, 68 in the mandibular part, and 11 in the region extending from maxillary to mandibular area. More 70% of the cases were fibroma originating in the molar area.
Radioisotopic methods are widely applied to investigations of bone sarcoma and soft tissue neoplasms. We have at our disposal molecules with osseous, tumoral or vascular tropism. Their use, as single agents or combination, is helpful in positive and differential diagnosis and provides nosological informations. They are also useful in treatment monitoring and in long-term follow-up.
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We describe a hemorrhagic chondroid chordoma involving the sella turcica with suprasellar extension. The CT and MRI appearances mimiked a hemorrhagic pituitary adenoma. Chondroid chordoma is a variant composed of elements of both chordoma and cartilaginous tissue. An uncommon bone neoplasm, located almost exclusively in the spheno-occipital region, it is usually not considered in the differential diagnosis of a tumor with acute hemorrhage in the sellar region. We discuss the clinical and radiological characteristics which may allow one to differentiate chondroid chordoma from other tumors of this area.
Neoplasms of the foot are relatively rare problems. Because of their rarity, many physicians are not familiar with their diagnosis or treatment. In addition, there is a diversity of opinions about the histogenesis, nomenclature, and treatment of these tumors. A systematic approach to evaluation, diagnosis, and treatment is necessary to ensure prompt, appropriate management of these problems. Topics discussed in this article included the initial evaluation, staging, biopsy, surgical resection, and postoperative care.
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Second primary cancers were studied in persons with rare tumors between 1943 and 1980. The risk of developing a new cancer was evaluated in 7,211 persons with cutaneous melanoma, 1,784 persons with eye cancer, 10,273 persons with tumors of the brain and nervous system, 1,935 persons with thyroid cancer, 1,542 persons with bone tumors, and 2,318 persons with malignant neoplasms of the connective tissue. All cancer patients were diagnosed in Denmark between 1943 and 1980 and survived for 2 or more months. Nonmelanoma skin cancers were excluded from the analysis, whereas tumors of the brain and nervous system included both benign and malignant neoplasms. Overall, patients with these cancers showed no greater incidence of new tumors than expected from comparisons with the general population. An excess of chronic lymphocytic leukemia was observed subsequent to all cancers derived from the neural tube, i.e., melanoma and tumors of the eye, brain, and nervous system. Bone cancer occurred excessively, although the possibility of misclassified metastases could not be eliminated. Patients with tumors of the brain and nervous system who survived for 10 or more years developed significantly more cancers of the kidney and connective tissue and melanoma than anticipated. A deficit of second cancers of the digestive system was noted after primary bone and connective tissue cancers, in contrast to an excess of second cancers of the lung and kidney. Although based on few cases, patients with bone cancer showed a large excess of eye cancer as a second primary. The association between cancers of the breast and connective tissue was found to be bidirectional. Persons with connective tissue cancer were at increased risk of developing non-Hodgkin's lymphoma. Thyroid cancer patients were at high risk of subsequent tumors of the brain and nervous tissue and non-Hodgkin's lymphoma. However, contrary to previous reports, the risk of breast cancer was not elevated following thyroid cancer.
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The authors present an immunohistochemical analysis of tissue from five cases of morphologically distinctive Ewing's sarcoma in bone and soft tissue. The mean age of the five patients was 16.6 years, with a range of 6-28 years. The tumors existed in chest wall, pelvis, and lower extremities. Two siblings with tumor in bone were clinically diagnosed as having Ewing's sarcoma. All cases had immunoreactivity for neuron-specific enolase (NSE), and four cases revealed positive staining for Leu-7. Neuron-specific enolase is highly specific for neurons and neuroendocrine cells. In addition, immunoreactivity for Leu-7, expressing a natural killer activity, has been demonstrated in peripheral nerve fibers and neuroendocrine cells. The authors suggest that NSE and Leu-7 immunoreactive small round-cell neoplasm is probably a primitive neuroectodermal tumor and should be categorized as Ewing's sarcoma in bone and soft tissue.
Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma in adults. MFH occurrence in the bones of the skull is extremely rare, and, to our knowledge, it has not been reported in a patient with a history of craniotomy. A 69 year old woman presented with a large mass in the left fronto-temporal bone. The patient's medical history included a left fronto-temporal craniotomy 8 years prior to the present admission for neck clipping of an aneurysm of the left middle cerebral artery. Following preoperative work up, the skull tumor was resected, and pathology showed that the mass was composed of spindle-shaped fibroblastic cells arranged in a storiform pattern, and of numerous multinucleated giant cells. A diagnosis of MFH was established. The patient died of intracranial hemorrhage from a subsequent tumor 22 months after resection of the first tumor.
An analysis of 6 representative cases of tumors of the pelvis and lower extremities demonstrates the clinical usefulness of computerized tomography (CT) in preoperative evaluation. CT provides information regarding precise size of the mass, its location and relationship to muscle bundles and fascial planes, definition of the margins of the mass, relation to neural, vascular and bony structures not previously available by plain film and angiography. This information is of particular value to the orthopedic surgeon in evaluating the feasibility of en bloc resections of tumors and in planning reconstructive procedures.