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Chronic ulcers of the leg are common in Brazil, perhaps more common than in the developed world. We report a case of a chronic ulcer of the leg following extensive scarring due to a bite by a venomous snake, which eventually led to a squamous cell carcinoma.

A 51-year-old native of Rio de Janeiro presented with bilateral synchronous Paget's sarcomas in the tibiae, which developed in the upper right tibia and in the distal third of the left tibia. There were no other areas of Paget's disease. The largest tumor spread to the right inguinal nodes and also soft tissue. The tumor in the left tibia spread dramatically in the soft tissues up the leg and only involved the medullary cavity in its inferior portion. The patient died, but there was no autopsy. Comments are made about the prevalence of Paget's disease in Rio de Janeiro.

Mazabraud's syndrome, though uncommon, is reported increasingly frequently. It represents an entity readily recognisable radiologically on MR imaging. Awareness of the syndrome, particularly when the myxoma is solitary, can prevent misdiagnosis of intramuscular myxomas (especially when large) as malignant mesenchymal tumors containing myxoid tissue. We review the 34 cases previously reported in the literature and include a recent case from our center.

A bulky tumor of the first lumbar vertebra is described. The case is the 21st to be reported. The tumor resembled an aneurysmal bone cyst radiologically. It was resected without incident. The previously reported cases are reviewed and the literature discussed.

We summarize our experience with 238 cases of Langerhans cell granulomatosis (LCG), 198 of whom were followed for a median period of 10.5 years. Our patients did well unless overtreated, and no deaths were attributed to the disorder itself. The disease may appear in unifocal or multifocal form, and treatment is based on this fact. Virtually all patients recovered completely except for occasional residual orthopedic problems or residual diabetes insipidus. Several of the patients underwent subsequent pregnancies without difficulty. The granulomas primarily occur in bone, but lung, skin, and lymph nodal involvement is not uncommon. Involvement of thyroid, thymus, and other sites is rare. The hallmark of the disease is the accumulation of Langerhans cells (LCs). We review the pathology of LCG by histology, electron microscopy, and immunolabeling. LCs originally were identified in squamous epithelium, but these cells are part of the widespread system of dendritic cells. The latter cells, which arise from CD34+ progenitors, are specialized and efficient antigen-presenting cells for T-cell-mediated immunity. In LCG, however, the major associated cells are not T cells, but mature eosinophils: hence the original name eosinophilic granuloma. Confusion about terminology has been based upon the scanty and rather crude pathology reports in the original literature. The term histiocytosis X was meant to cover a spectrum of three diseases--eosinophilic granuloma, Hand-Schüller-Christian disease (HSC), and Letterer-Siwe disease (LS)--but HSC and LS have no basis in pathology and hence the terms are meaningless. The term HSC has become a synonym for multifocal eosinophilic granuloma (LCG). The term LS has been used in reporting a number of benign, malignant, or unknown conditions. We prefer the term LCG to avoid confusion with the term histiocytosis X because there is evidence that the LC is not a member of the mononuclear phagocyte system and hence not a tissue macrophage, and because the use of the term "histiocyte" has become a convenience in much of the literature when reporting incompletely understood diseases.

To study the imaging features of patients with chronic ulcers of the leg that were associated with malignancy.All patients who on biopsy were proven to have malignancy--the majority of which were squamous cell carcinoma-were included in a prospective study. Ulcers limited to the foot were excluded but ulcers of the leg which extended into the foot were included. Amputation was performed in all but two patients, due to pain, bleeding or tissue necrosis.The etiology was multifactorial. The mean duration of the ulcers was 36 years including venous ulcers, extensive scarring of the leg secondary to infection, injury or burns. One ulcer was secondary to a snake bite. The remainder, usually in the upper part of the leg, had repeated episodes of blunt trauma or knife wounds, which were also complicated by infections which failed to heal or, if they healed, regularly recurred. Although arterial insufficiency was not primary in any patient, most were of advanced age and it may have been an element in some patients. Despite infection, osteomyelitis was present in only one patient. The essential features were bone destruction, soft tissue mass and periosteal reaction. The bone destruction was visible on the radiographs in all but one case. The soft tissue masses varied in size but in general were very large. The periosteal reaction varied in type but most commonly was lamellated. The classic undulating solid periosteal reaction of venous stasis was only occasionally present. The periosteal reaction was nonspecific in the majority of cases and did not aid in the diagnosis or etiology. MRI and CT studies were performed in six patients. These were helpful in defining the extent of bone destruction and periosteal reaction but were not essential in management.Chronic ulcer present for decades that then undergoes malignant change is a disease of developing countries where patients only consult physicians when they have developed complications such as pain, bleeding or tissue necrosis. Chronic ulcers may require to be biopsied at regular intervals as malignant change in these ulcers is directly related to their duration.

We present a unique case of metastatic leiomyomatosis to the skeleton. The very extensive involvement of the axial and peripheral skeleton with "ring" lesions and associated cyclical premenstrual pain eventually led to the correct diagnosis and total relief with hormonal therapy.

We have reported the case history of a 72-year-old woman who was on hemodialysis for 15 years. Her course was marked by many of the musculoskeletal complications of ESRD including CTS, stromal amyloid deposition of synovium, amyloid cystic degeneration of bone, and inflammation of the synovium due to the deposition of calcium oxalate and calcium pyrophosphate microcrystals. She also had evidence of metabolic bone disease: moderate osteoporosis related to secondary hyperparathyroidism and osteomalacia related to aluminum deposition at the mineralization front. The pathological and radiological findings associated with her bone disease are described.

Sixty-three patients with osteogenic sarcoma of the long bones, all of whom were treated with chemotherapy, demonstrated striking and unusual radiographic changes. Patients with a "good" radiographic response (48%) showed the most dramatic changes, including medullary sclerosis, prominent periosteal new bone formation, and disappearance of the soft-tissue mass; and these findings correlated well with the histological grading of the surgical specimens. Radiographic evaluation of patients receiving chemotherapy for osteogenic sarcoma is a valuable method of assessing response.

In a review of more than 1000 patients with osteogenic sarcoma of bone, the vertebral column was the site of the primary tumor in 10 patients. The vertebral osteogenic sarcoma in 4 of the 10 patients was secondary; to Paget's disease of bone in 3, and to irradiation received for a prior cancer in 1. Frequently, cord compression caused severe neurologic symptoms. Prognosis was always poor. Seven patients died of their disease within a year of histologic diagnosis. The differential diagnoses between osteogenic sarcoma and three benign tumors are reviewed, osteoblastoma being the tumor most frequently confused with osteogenic sarcoma. The radiologic and histologic criteria for distinguishing between the two are discussed.

Osteonecrosis of the femoral and humeral heads is a serious complication of therapy for Hodgkin's disease and non-Hodgkin's lymphoma. Twenty-five patients were reassessed 5 years after being initially reported, in order to study the further progress and natural history of this complication. In addition, six recent patients who have also developed this condition are presented. With 5-year additional follow-up, no patient had developed symptoms of osteonecrosis in any bone other than those initially involved. Five patients developed severe complications thought to arise from their therapy suggesting that this group of patients were more sensitive to radiation injury than other patients treated with this modality. During the relatively short follow-up 5-year period, a surprising finding was the fact that 31% of the patients with Hodgkin's disease and 50% with non-Hodgkin's lymphoma had died. It is recommended that patients treated for lymphoma with steroid containing chemotherapy and radiotherapy be observed carefully for the occurrence of joint pain. Early diagnosis should lead to attempts to prevent total joint destruction.

Sixty patients with sacrococcygeal chordoma, who were seen at this center between 1946 and 1985, were studied with particular attention to the radiographic findings. This study was undertaken because of the large number of these cases and comparison was made between the plain films available in 39 patients and the computed tomography CT studies in 22. Bone destruction was found in 78% on plain films but in 90% on CT. A soft tissue mass was identified in plain films in 60% but in 90% on CT. Calcific debris was found in plain films in 44% but in 87% on CT. Mostly the debris consisted of coarse irregular fragments and probably represented sequestrated necrotic bone. Myelography was performed in only 15 patients. Angiography was studied in 10 cases. Of the 60 patients 88% underwent surgical resection. The tumor recurred in 80% and in only 20% was there no evidence of recurrence. Distant metastases occurred in 24% of patients. Fifty percent survived 5 years; 28% survived 10 years; mean survival 7.5 years.

A retrospective study of 25 patients with malignant lymphoma who had osteonecrosis of either the femoral or humeral head(s) was undertaken. The common factor present among all patients was the administration of intermittent steroid-containing combination chemotherapy. Seventeen Hodgkin's disease patients received chemotherapy predominantly consisting of an alkylating agent, vincristine, procarbazine, and moderate amounts of prednisone. The non-Hodgkin's lymphoma patients were on various moderate dosage steroid-containing protocols, except three who received prolonged high-dose steroid-containing chemotherapy regimens. Sixteen of the 17 Hodgkin's disease patients and five of the eight non-Hodgkin's lymphoma patients received radiotherapy to the bones that subsequently developed osteonecrosis. Two of the three non-Hodgkin's lymphoma patients who were not irradiated were treated with high-dose steroid-containing chemotherapy regimens. Symptoms developed in patients 12 months to 32 months after completion of chemotherapy and radiotherapy, respectively. Osteonecrosis was a long-term complication of treatment between 1970 and 1979 and occurred in 1.6% of the Hodgkin's disease and 0.12% of the non-Hodgkin's lymphoma patients treated. The authors conclude that the patients at highest risk for this complication are those who receive both radiotherapy to the affected bone(s) and intermittent steroid-containing multiple drug chemotherapy.

Cas particulier d'un chordome sacroccoxygien sans destruction osseuse, traite par chirurgie puis radiotherapie. Diagnostic differentiel avec un sarcome retrorectal

Seven patients after treatment of Hodgkin's Disease who developed bone sarcomas in the radiation field were seen at this centre over the past eight years. Radiation-induced sarcoma in bone in patients with Hodgkin's Disease is poorly documented. The large number of cases appeared to be an important new development and led to our review of all the patients with radiation-induced sarcomas (RIS) seen at this centre over the past 40 years. Thirty-seven patients with RIS in previously normal bone were found, and of these, only one patient with underlying Hodgkin's Disease, who was seen here 27 years earlier. Hodgkin's Disease and breast cancer were the most common primary underlying conditions and, as a result, the bones of the shoulder girdle were the commonest site of radiation-induced sarcoma. The clinical histories and radiographic findings of the eight patients with underlying Hodgkin's Disease are discussed in detail.

Renal cell carcinoma (hypernephroma, renal adenocarcinoma) metastasizes to the skeleton in approximately 10% of cases and characteristically produces expansile lytic lesions (Forbes et al., 1977). Although sclerotic changes may be seen following radiotherapy, osteosclerotic metastases in this condition prior to therapy are virtually unknown (Forbes et al., 1977; Sherman and Pearson, 1948). We report here well-documented case of renal cell carcinoma which produced osteoblastic metastases and was confirmed by autopsy examination.

The authors describe a case in which a delayed diagnosis of an absecess in the right upper quadrant (secondary to a ruptured gallbladder) was caused by a mistaken diagnosis of interposition of the colon between the liver and the diaphragm on a computed tomogram scan and the lack of significant clinical symptomatology. This case report is presented hopefully to enable one to suggest an earlier diagnosis of a similar situation and to illustrate additional pathology on computed tomography scans.

Primary and secondary bone tumours usually result in areas of increased tracer uptake on bone scans because of osteoblastic reaction of the bone surrounding the tumour (Kirchner, 1980). Rarely, tumours may result in areas of reduced tracer uptake (Georgen et al, 1974; Sy et al, 1975; Goris et al, 1980; Kado et al, 1980; Bushnell et al, 1983; Makhija, 1983; Weingrad et al, 1984). In this report, a case of osteosarcoma with prominent fibroblastic reaction is presented as it resulted in the unusual finding of a “cold” lesion on bone scintigraphy.

Three cases of cerebellar ectopia first producing symptoms in adult life are reported. This potentially remediable anomaly may not be suspected in adults, in whom associated congenital bony abnormalities of the skull and cervical spine are often absent. In these cases diagnosis depends on radiological contrast studies; in particular it is important to examine the cervical canal in the prone and supine positions.

Involvement of the spine by chondrosarcoma is rare. Three recent cases involving the cervical with or without extension to the thoracic spine are reported. Detailed radiologic investigations to evaluate the extent of the disease are essential to management. Computerized axial tomography and angiography were performed in 1 case and were extremely helpful. Surgical resection of the lesion is the only currently effective method of treatment, but long-term survival figures are not good.

Fifty eight patients seen at Memorial Sloan-Kettering Cancer Center over a 50-year period were reviewed for lesions of the clavicle. A variety of malignant neoplasms, benign neoplasms, and tumor-like lesions were seen. There were 30 malignant neoplasms, the commonest of which were plasmacytomas, osteosarcomas, and Ewing sarcomas. There were five postradiation sarcomas. An unusual granulocytic sarcoma with dense sclerosis was described. Among the benign lesions were two osteochondromas, two hemangiomas, and a giant cell tumor secondary to Paget disease. The tumor-like lesions included six aneurysmal bone cysts and five eosinophilic granulomas. There were two patients with chronic sclerosing osteomyelitis and one each with sternocostoclavicular hyperostosis (Sonozaki syndrome) and condensing osteitis. An unusually large lesion of pseudogout and a large brown tumor were also included in the series.

Fifty cases of lower extremity tumors examined with computed tomography were reviewed. Of the 50 cases, 41 had gross pathologic correlation, either by enbloc resection (26) or by amputation (15). Important information regarding size, location, definition, and anatomic relation of tumors to vital structures (neural, vascular, and osseous) may be ascertained by this means with great accuracy. Localization of vessels by intravenous infusion of contrast material during the scan, or after arteriography with intraarterial contrast infusion, is found to enhance such evaluation. This information is particularly important where the feasibility of en-bloc resection is being evaluated.

Sixteen cases of radiogenic malignant bone tumors which developed in patients 4 to 31 y after they had received radiation therapy for Hodgkin's disease are compared with 70 similar cases occurring after radiation exposure for other causes. No significant differences in age at irradiation, latent period, histology of tumors or radiographic characteristics were found between the Hodgkin's cases and those of the comparison series. Fourteen of the 16 tumors in Hodgkin's patients, or 87%, were diagnosed in the 15-y period between 1971 and 1985 in contrast to only 34% of the larger non-Hodgkin's series. This difference appears significant and is associated with a doubling of the proportion of Hodgkin's patients who are now living past the minimum latent period for such tumors. The median dose in the Hodgkin's patients, 4000 cGy, is less than the 5100-cGy median in the other patients, and the range and protraction of the total doses are much less. In Hodgkin's patients who have received total nodal irradiation, the volume of osseous tissue which is exposed may reach 25% of the total in the body. This is much greater than in most other treatment plans. Six of the Hodgkin's patients received chemotherapy within a few monthsmore » of irradiation; only three of 70 non-Hodgkin's patients did so. All of the last factors may have modified the risk of radiation carcinogenesis in the Hodgkin's patients but the data are not yet adequate for quantitation of the effect.« less

Two adult patients who had synovial chondromatosis occurring within bursae formed around osteochondromas are described. In spite of marked cellular atypia of the cartilage cells, the clinical course was unremarkable, pointing out the discrepancy between histologic changes and subsequent behavior in this condition. Histologic features, in addition, are not always helpful in distinguishing primary from secondary chondrometaplasia.

Aneurysmal bone cysts have been found in virtually every bone in the body (Dabska and Buraczewski, 1969; Lichtenstein, 1957; Jaffe, 1958). The most common locations are the long bones and the spine which account for about 75% of all lesions. They are relatively rare in the pelvis (Tillman et al., 1968). The majority of aneurysmal bone cysts are treated either with curettage alone or curettage combined with cryosurgery (Mufti, 1978; Peltier and Jones, 1978). Although some may recur, surgery alone or in combination with irradiation has produced good results (Dahlin et al., 1955; Tillman et al., 1968; Slowick et al., 1968). Radiation therapy alone is used for lesions where surgery is not possible or where it would produce serious impairment of function (Marks et al., 1976; Lichtenstein, 1957). Radiation therapy of aneurysmal bone cysts has been reported by Nobler et al. (1968) to be superior to surgery in a small series of patients. Little is known about the dose necessary to produce permanent control of the...

Osteocartilaginous exostoses (osteochondromata) are rather common bone tumors. Although most are straightforward lesions, one may occasionally encounter one of a number of well-recognized complications. In the current case, secondary malignant transformation and bursal sac formation were present. Additionally, numerous malignant chondroid nodules were shed into the attached bursa, giving rise to a large soft tissue mass, a situation which we feel is both remarkable and, to our knowledge, unique

Sarcomas rarely follow irradiation of benign lesions of bone as the dangers of this form of therapy is well recognised. Forty-three patients with soft tissue neoplasms — mainly carcinoma — were irradiated and developed sarcomas in the radiation field. Twenty-four of these patients were seen at this centre in the last decade, a considerable increase compared to earlier years. Radiation osteitis may indicate the nature of the tumour but was only present in 50%. A purely lytic or purely sclerotic pattern was commoner than a mixed lytic and sclerotic appearance. CT scan, bone scan and angiography were helpful in delineating disease but did not assist in diagnosing the type of tumour. A tumour arising within the irradiated field is an indication for biopsy. Prognosis of radiation-induced sarcoma (RIS) remains poor, only five of the 43 patients survived 5 years.

We report 16 cases seen in the Memorial Sloan-Kettering Cancer Center (MSKCC) during the past 50 years. These patients had been treated with external radiation for Hodgkin disease and had developed sarcomas in the field 4–31 years after the diagnosis of Hodgkin disease. Most of the tumors (12 of 16) occurred in the chest wall. There were three tumors of the pelvis and an unusual osteosarcoma of the femur following treatment for a primary Hodgkin disease of the femur. The tumors were predominantly osteosarcomas (9). In addition, there were five malignant fibrous histiocytomas, one fibrosarcoma, and one chondrosarcoma. Prognosis was poor; the mean survival was 12 months. Survival of patients with other primary cancers who developed radiation sarcomas was not significantly different from that of patients with Hodgkin disease. Hodgkin disease is now the most common tumor among radiation-induced sarcomas in previously normal bone and has surpassed breast cancer, which was previously the most common original tumor.

Two cases of unusually large bone islands are presented. Characteristic rounded discrete densities were seen in the left supra-acetabular area and the left ilium, respectively. The major importance in recognizing bone islands is the need to distinguish them from other pathological processes including osteoblastic metastases, osteoid osteoma, and osteomas.