Case report 434. Primary mediastinal endodermal sinus tumor with skeletal metastases.
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Biomedical subjects
Publications and source records attributed to W Mnaymneh.
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Solitary osseous myeloma is an uncommon malignancy of bone which is distinguished from multiple myeloma by being localized and without associated monoclonal gammopathy or Bence-Jones proteinuria. This disease may present with a progressive symmetrical sensorimotor neuropathy. A recent case of localized myeloma with polyneuropathy is presented along with a review of the literature pertinent to orthopedic surgeons.
The usefulness of indium-111 white blood cell scintigraphy in the diagnosis of subacute or chronic bone infection was examined in 21 orthopedic patients. In-111 WBC imaging was compared with gallium-67 and technetium-99m methylene diphosphonate skeletal scintigraphy and bone radiography, all studies being performed within 1 week. In-111 WBC scintigraphy showed no definite advantage over Ga-67 scintigraphy in the identification of chronic bone infection. The two tests had the same sensitivity (80%) and similar specificity (In-111 WBC 75%, Ga-67 83%; difference not significant). Bone radiography had a sensitivity of 60% and a specificity of 67%. A negative Tc-99m MDP bone scintigram ruled out infection (sensitivity 100%), but because of low specificity (25%), final evaluation required performance of Ga-67 or In-111 WBC scintigraphy.
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Hemipelvectomy is a lifesaving procedure when used appropriately and yields a 35%, five-year survival in the cancer patient. However, the standard posterior flap for closure is not always available. Two flaps, the thigh flap and the rectus abdominis myocutaneous flap, are described in which any soft tissue defect resulting from hemipelvectomy can be safely closed primarily when the posterior flap is not available. The total rectus abdominis flap is previously undescribed and unique in its application. These techniques significantly contribute to the surgeon's armamentarium for decreasing morbidity and mortality and resectability of unusual pelvic and thigh tumors.
Six cases of malignant fibrous histiocytoma (MFH) primary in bone were studied clinicopathologically with ultrastructural study of three. The histologic differentiation of MFH from osteosarcoma and from fibrosarcoma is discussed. Two of the six cases had highly malignant tumors histologically designated as Grade III and IV; one died with diffuse metastatic disease eight months after diagnosis and the second developed pulmonary metastases within ten months. Review of the literature revealed that 45 of the total 119 reported cases had been followed for less than five years; these cases were considered unevaluable for assessing biologic behavior. The remaining 74 cases, followed for more than five years or until death, showed a 36.5% five-year survival rate. All but one of the cases which developed metastasis within five years did so within the first two years. It is therefore concluded that MFH of bone is a rapidly metastasizing tumor with a relatively low five-year survival rate. Paradoxically, 13 of these 74 cases (17.6%) survived between five and 38 years with no evidence of disease. This is comparable to the long-term survival rate in osteosarcoma. The five-year survival rate is also comparable to that of fibrosarcoma of bone.
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A 32-year-old man suffered sharp trauma to the volar aspect of the wrist. A lacerated tendon found on exploration had been left unrepaired and the skin closed primarily. A few months later the patient presented with a nodule at the same site. Reoperation demonstrated a 2 X 1 cm nodule bridging the gap between the stumps of the previously severed palmaris longus tendon. Pathologic examination of the nodule revealed the typical microscopic appearance of giant cell tumor of tendon sheath (GCTTS) in continuity with the lacerated tendon. The literature regarding the relationship of trauma to the development of GCTTS presents interesting controversy about whether this lesion is reparative or neoplastic.
A 73-year-old man developed a solitary metastasis to the cuboid bone of the foot one year after a left upper lobectomy for bronchogenic adenosquamous carcinoma. Irradiation controlled the pain for 1 1/2 years. Metastatic work-up failed to show other foci; hence, a Syme's amputation was performed. The patient remains well with no evidence of disease 3 1/2 years after biopsy diagnosis. Metastasis to the peripheral bones of the feet are extremely rare. Only 23 biopsy-proven cases are recorded in the English literature, seven of which were of bronchogenic origin. Most of the cases were part of disseminated metastatic disease, with the longest reported survival being 26 months. The present case had an apparently solitary metastasis, with a relatively long survival of 44 months following diagnosis.
An anusual pathologic fracture of clavicle was caused in a 48-year-old woman by an arteriovenous malformation. The lesion radiologically mimicked a neoplastic process. Arteriography disclosed the vascular cause. Treatment was achieved by interrupting the feeding vessels and resecting the involved bony segment.
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We have described an unusual case of giant cell tumor of the ischium. The remarkable features of the case are that it occurred in a relatively rare site and that it transformed radiologically from a localized, well circumscribed lesion into a very destructive lesion over a period of several months, though histologically it remained benign in appearance. The lesion was successfully treated by a modified hemipelvectomy. We emphasize that the treatment of choice in giant cell tumor of bone is total en bloc excision and not curettage or irradiation.
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