Case report 598: Os centrale carpi.
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Biomedical subjects
Publications and source records attributed to A Greenspan.
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Idiopathic periosteal hyperostosis with dysproteinemia has been recognized as a separate entity since its description by Goldbloom and colleagues in 1966. Very few cases have been reported to date, and the radiologic literature is very scanty regarding this syndrome. This article reviews the entity and illustrates it with our own example.
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A 35-year-old caucasian man developed mild and transient signs of chronic graft-versus-host disease (GVHD) 5 months after bone marrow transplantation. At 16 months he presented with painful cramps in hands, feet, and truncal muscles. Electrophysiological studies revealed generalized sensory neuropathy. There was occasional fasciculation and high frequency motor units during involuntary muscle contractions, and agonist/antagonist cocontractions of peripheral muscle groups (non-continuous). A sural nerve biopsy showed Schwann cells and macrophages with myelin debris, and Schwann cell stacks with collagen pockets. The patient's symptoms responded to treatment with glucocorticoids and azathioprine. We propose that this patient's incapacitating polyneuropathy was a major manifestation of chronic GVHD, suggesting that the peripheral and possibly central nervous systems can be targets of chronic GVHD.
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Eighteen patients with advanced measurable breast cancer were treated with elliptinium acetate 100 mg/m2 x 3 days every 3 weeks. Fourteen of these patients had failed prior chemotherapy. Two patients had an objective tumor response of greater than 4 weeks. Myelosuppression, renal insufficiency and thrombophlebitis were rarely encountered and alopecia was not seen at all. This study demonstrates that elliptinium has minimal activity in recurrent breast cancer with a favorable toxicity profile.
Dysplasia epiphysealis hemimelica (Trevor-Fairbank's disease) is a rare anomaly that must be differentiated from other epiphyseal and periepiphyseal abnormalities. Computed tomography is extremely useful in diagnosing and defining the extent of this dysplasia, as well as in assisting the surgeon in planning definitive treatment.
Currently, determining whether a cartilage tumor is benign or malignant is a challenge. This article explains the available diagnostic tools and the tumor characteristics used to make such judgements. Treatment of chondrosarcoma is also discussed.
In a phase I study 28 patients with lymphohematopoietic malignancies were treated with escalating doses of etoposide combined with cyclophosphamide 120 mg/kg and either fractionated total body irradiation (TBI) 1320 cGy in 11 fractions (n = 17) or busulfan 16 mg/kg (n = 11). Twenty transplants were allogeneic, seven autologous and one syngeneic. The maximally tolerated dose of etoposide in combination with TBI and cyclophosphamide was 60 mg/kg; at etoposide doses of 65 mg/kg three patients developed life-threatening or fatal mucosal toxicity. In combination with busulfan, the maximally tolerated dose of etoposide was 30 mg/kg. At an etoposide dose of 40 mg/kg two patients developed life-threatening or fatal toxicity (one mucosal, one hepatic). Mucositis requiring narcotic analgesics, hepatotoxicity, hematologic toxicity and interstitial pneumonitis were otherwise similar in TBI and busulfan-treated patients. Skin toxicity was observed significantly more often in the busulfan group. Five deaths occurred before day +30, two in the TBI group and three in the busulfan group. Eleven patients are surviving, ten in continuous complete remission, at a median of 9 (range 3-23) months following transplantation. Etoposide in combination with TBI and cyclophosphamide or busulfan and cyclophosphamide is associated with significant but acceptable toxicities. The maximally tolerated dose of etoposide is higher when combined with TBI than with busulfan. Promising response rates in this high risk group of hematologic malignancies warrant further evaluation of these etoposide containing conditioning regimens.
The ischium represents one of the rarest sites of giant cell tumor of bone. Two additional cases are presented here. The unusual site, aggressive appearance and occurrence in older individuals are suggestive of other primary and secondary malignancies. Characteristic features of giant cell tumor and current treatment options are reviewed.
Twenty-two histologically proven cortical metastases in 11 patients with bronchogenic carcinoma (10 squamous cell and one adenocarcinoma) were studied. The intracortical foci could be classified according to four distinctive patterns of bone destruction: small focal intracortical lesions ("cookie-bite" or "cookie-cutter" pattern); large osteolytic lesions; saucerized intracortical lesions with well-defined periosteal reaction; and lesions with predominant cortical destruction extending into the soft tissue as well as the medullary cavity. All lesions were located in the femoral bones.
Trispiral tomography is an x-ray technique that is essential in the diagnosis and management of a wide variety of problems involving the carpal bones and their joints. It facilitates the visualization of acute fractures, particularly those of the scaphoid and hook of the hamate, as well as cysts, sclerosis, and erosive lesions. It is useful in differentiating between acute and chronic changes, and it is often indispensable in evaluating healing after acute fractures or bone grafts to treat nonunions.
Five of 55 patients with metastatic carcinomas or unresectable primary carcinomas developed a peripheral neuropathy with "low dose" cisplatin therapy, consisting of 20 mg/m2 weekly. The neuropathy was primarily sensory, consisting of painful parasthesias and numbness in a "stocking glove" distribution. The cumulative dose at which neuropathy developed varied from 100 to 640 mg/m2. The symptoms and signs of neurologic dysfunction were minimally reversible despite discontinuation of the drug. Despite lower dose delivery of cisplatin, significant debilitating neurologic toxicity may occur.
The use of arthrotomography and postexercise spot films has increased the effectiveness of wrist arthrography in demonstrating triangular fibrocartilage abnormalities.
A case of clear-cell chondrosarcoma occurred in the proximal femur of a 32-year-old man. The tumor was complicated by a focus of myositis ossificans resulting from a previous intralesional biopsy. Histologic findings included an unusual marrow infiltration by the tumor. The patient was treated with wide resection and has no evidence of local recurrence or distant spread of disease 18 months later.
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For the past 4 years, the authors have used the radial head-capitellum view in 150 patients being examined for trauma to the elbow. It has proved valuable especially in the evaluation of the radial head, the coronoid process, and the capitellum.