Mixofibrosarcoma de bajo grado de la pared torácica / A low-grade myxofibrosarcoma of the chest wall

El mixofibrosarcoma es una neoplasia maligna infrecuente, que puede originarse en los tejidos blandos, se lo ha estadificado como de alto o bajo grado y la localización más frecuente son los miembros inferiores. El reconocimiento clínico del mismo es dificultoso, en primer lugar porque la clínica de otros sarcomas de partes blandas es similar y además semejan lipomas o aún quistes. La extirpación quirúrgica amplia es el tratamiento de elección, por la propensión de este sarcoma a la recidiva local. Los catalogados como de alto grado en un 30 % de los casos, pueden originar metástasis a distancia, especialmente a hueso, pulmón y ganglios linfáticos. La histopatología asegura el diagnóstico en la mayoría de los casos. Comunicamos el caso de un mixofibrosarcoma de bajo grado, en un hombre de 43 años, que a los tres años de control evolutivo, luego de la resección quirúrgica, no presenta recaída local ni distante.

Myxofibrosarcoma is an uncommon soft tissue sarcoma that is grading as low or high malignancy. The principal sites of involvement are the lower limbs. Clinical recognition is difficult since it resembles a cyst, a lipoma, other soft tissue sarcoma or even benign conditions as panniculitis. The wide surgical excision is the main therapeutic approach because local recurrence is frequent. The high grade variant is associated in 30 % of the cases with distant metastasis, especially to the lung, bone and lymph nodes. The histopathological features allow an accurate diagnosis in most cases. A 43 year-old man with a myxofibrosarcoma of the chest wall is reported with a follow-up of two years without recurrence.

Estudio experimental de la interfase oseointegrada

El estudio del material del implante en relación con el tejido vivo (hueso y/o médula) es de especial importancia. En esta presentación deseamos resumir una serie de datos experimentales obtenidos del estudio de diferentes materiales y condiciones que han posibilitado disponer de una metodología que puede ser aplicada sobre un número significativo de muestras, obteniéndose medidas y datos a nivel microscópico y con valor estadístico

Human leukocyte antigen matching, radiographic score, and histologic findings in massive frozen bone allografts.

Forty-six patients receiving frozen bone allografts, preoperatively tissue typed for human leukocyte antigen and ABO antigens, were radiographically evaluated according to the Musculoskeletal Tumor Society scoring system at a mean followup of 55 months. Patients who matched for 1 or 2 Class I human leukocyte antigens with the donor scored higher than patients totally mismatched, but differences were not significant. Matching for Class II human leukocyte antigen and ABO antigens seemed not to influence radiographic outcome of allografts. In sixteen patients histologic specimens were obtained. Five of 16 patients who showed histologic parameters of an immune response scored significantly lower than those who did not. Processed frozen bone allografts, because of their lack of viable donor cells, most likely trigger an indirect pathway of alloantigen recognition in the recipient. This type of recognition may generate in the recipient either a chronic type of rejection or an immunologic state of tolerance to grafted antigens that cannot be measured with human leukocyte antigen blood tests. This may explain difficulties in correlating human leukocyte antigen mismatches between the donor and recipient with frozen bone allograft performances.

Estudio experimental de la interfase oseointegrada

El estudio del material del implante en relación con el tejido vivo (hueso y/o médula) es de especial importancia. En esta presentación deseamos resumir una serie de datos experimentales obtenidos del estudio de diferentes materiales y condiciones que han posibilitado disponer de una metodología que puede ser aplicada sobre un número significativo de muestras, obteniéndose medidas y datos a nivel microscópico y con valor estadístico

Osteoid osteoma of the hip. Percutaneous resection guided by computed tomography.

Seven patients with a presumptive diagnosis of osteoid osteoma located at the hip were treated with percutaneous resection of the nidus through computed tomography guidance. Histologic confirmation was obtained in 5 of the 7 patients. The average hospital stay was 27 hours. At followup, from 12 to 40 months, all patients remain asymptomatic. This procedure presents potential advantages that traditional open surgery techniques do not have.

Tumores musculoesqueléticos de la pelvis y la cadera / Soft tissue and bone tumores of the hip and pelvis

El tratamiento actual de los tumores musculoesqueléticos cuya filosofía es la reducción de la magnitud de la cirugía con reservación de los miembros y de ser posible de la función, ha provocado una gran demanda de los métodos del diagnóstico por imágenes. Cuando las lesiones asientan en la pelvis al tema etiológico se suma la dificultad en su detección debido a la particular anatomía del área. Con el objeto de analizar la incidencia de los tumores musculoesqueléticos y el aporte de divesos métodos diagnósticos se estudió una población constituída por 214 pacientes con tumores o lesiones seudotumorales pelvianas comprobados histológicamente. Se detectaron 19 lesiones seudotumorales, 27 tumores benignos, 6 tumores o células gigantes y 162 tumores malignos. Dos tercios de las lesiones seudotumorales y de los tumores benignos ocurrieron por debajo de los 30 años de edad. Los tumores malignos fueron mayoría (75 por ciento) destacándose las metástasis (70 por ciento de todos los tumores malignos). El tumor primario más frecuente fue el condrosarcoma. El diagnóstico por imágenes juega en la actualidad un rol significativo en el diagnóstico y estadificación de los tumores muescoesqueléticos. Junto con la clínica y la anatomía patológica constituyen el trípode indispensable para arribar al diagnóstico definitivo e instituir la terapéutica adecuada (AU)

Tumores musculoesqueléticos de la pelvis y la cadera / Soft tissue and bone tumores of the hip and pelvis

El tratamiento actual de los tumores musculoesqueléticos cuya filosofía es la reducción de la magnitud de la cirugía con reservación de los miembros y de ser posible de la función, ha provocado una gran demanda de los métodos del diagnóstico por imágenes. Cuando las lesiones asientan en la pelvis al tema etiológico se suma la dificultad en su detección debido a la particular anatomía del área. Con el objeto de analizar la incidencia de los tumores musculoesqueléticos y el aporte de divesos métodos diagnósticos se estudió una población constituída por 214 pacientes con tumores o lesiones seudotumorales pelvianas comprobados histológicamente. Se detectaron 19 lesiones seudotumorales, 27 tumores benignos, 6 tumores o células gigantes y 162 tumores malignos. Dos tercios de las lesiones seudotumorales y de los tumores benignos ocurrieron por debajo de los 30 años de edad. Los tumores malignos fueron mayoría (75 por ciento) destacándose las metástasis (70 por ciento de todos los tumores malignos). El tumor primario más frecuente fue el condrosarcoma. El diagnóstico por imágenes juega en la actualidad un rol significativo en el diagnóstico y estadificación de los tumores muescoesqueléticos. Junto con la clínica y la anatomía patológica constituyen el trípode indispensable para arribar al diagnóstico definitivo e instituir la terapéutica adecuada

Adamantinoma of the tibia masked by fibrous dysplasia. Report of three cases.

Three cases of adamantinoma of the tibia associated with and mimicking fibrous dysplasia or osteofibrous dysplasia are reported in children aged three, nine, and 16 years. The roentgenographic features were typical of intracortical fibrous dysplasia (osteofibrous dysplasia). These entities are not two distinct diseases, but rather are different histologic expressions of essentially the same process, which varies depending on location (predominantly intracortical or medullary) and age. Adamantinoma in children under ten years of age is not as rare as reported in the literature and was found in two of 14 cases in our files (14.3%). In some cases the fibrous dysplasialike component predominates over the scarce epithelioid islands of tumor cells and consequently is not recognized as adamantinoma. That may explain the frequent recurrences after incomplete excisions of supposed intracortical fibrous dysplasia lesions in young children. An extensive histopathologic study of the biopsy and/or surgical specimen by a specialized pathologist is therefore advisable.

Osteosarcomas arising on the surfaces of long bones.

Malignant bone-forming tumors that arise from the surfaces of long bones are far less common than those that arise from within bone. These surface osteosarcomas are clinically and radiographically similar, yet histologically they are quite distinct. In reviewing the literature, we classified the tumors according to three subgroups: parosteal (juxtacortical) osteosarcoma, periosteal osteosarcoma, and high-grade surface osteosarcoma. We also studied the clinical, radiographic, and histological findings in eighty patients who had been treated for an osteosarcoma over a forty-four-year period. Adequate follow-up data existed for forty-eight of the eighty patients. The duration of follow-up ranged from two to fifteen years after the initial operation (amputation or resection). Patients who had a parosteal osteosarcoma had the best prognosis; those who had a periosteal osteosarcoma, the next best; and those who had a high-grade surface osteosarcoma, the poorest. Because of these widely varying prognoses, the lesions require different treatment.

Metastases of carcinoma in the pagetic bone. A report of two cases.

Two men, aged 68 and 77 years, had metastases of carcinoma in pagetic bones. These cases were initially diagnosed clinically as Paget's sarcoma. Roentgenograms, scintigrams, and bone puncture biopsy specimens (BPBs) of the right scapula and the sacrum, respectively, showed only Paget's disease. However, an additional BPB obtained with the aid of computed axial tomography (CAT) revealed the presence of metastases of lung carcinoma in the right scapula and of a carcinoma of probable prostatic origin in the pagetic sacrum in the other. The observations testify to the usefulness of CAT-directed BPB when Paget's sarcoma is suspected, especially in cases located in the scapula, pelvis, or spine.

Spontaneous fractures of uninvolved bones in patients with Paget's disease during unduly prolonged treatment with disodium etidronate (EHDP).

Three patients with Paget's disease of bone were treated by disodium etidronate (EHDP) without interruption during periods of 18 to 30 months. In one case the daily dose was also unduly high (approximately equal to 18 mg/kg/day). A moderate to conspicuous diminution of the renal function was observed in all cases. The three patients developed skeletal pain in a gradual but progressively severe pattern. Seven nontraumatic fractures in nonpagetic bones were encountered. EHDP produces blockage of bone mineralization and excessive suppression of bone remodeling, therefore increasing the risks of fracture. EHDP affects not only pagetic bones but normal skeleton. A baseline evaluation of the renal function might help to identify those patients with greater risk to develop skeletal side effects during EHDP treatment.

Sarcoma complicating Paget's disease of bone. A clinicopathological study of 62 cases.

Out of 21 900 cases filed at the Latin-American Registry of Bone Pathology between April 1940 and July 1981, there were 987 with Paget's disease (4.51 per cent); 62 of these (6.28 per cent) were complicated by sarcoma and two were associated with giant-cell tumours of bone (osteoclastoma) without signs of malignancy. There was a slight predominance of men and the ages ranged from 45 to 87 years, with an average of 66 years. The most frequent sites were the femur (23 cases), the humerus (nine), the pelvis (10), and the tibia (nine). The low incidence of vertebral involvement (five cases) is noteworthy and is in sharp contrast to uncomplicated Paget's disease. The most common tumour type was osteosarcoma (39 cases), followed by fibrosarcoma (15 cases); other varieties (chondrosarcoma, malignant fibrous histiocytoma and reticulum-cell sarcoma) were much rarer. Most of the sarcomata occurred when the Paget's disease was polyostotic. Tumours often developed simultaneously, or at short time intervals, in the same or different bones; these bones had, in all cases, been affected by Paget's disease. The histological features of the osteosarcomata were characteristic, with large numbers of osteoclast giant cells, alternating with atypical osteoblasts, thus exaggerating the anarchic remodelling process of Paget's disease. The neighbouring areas of the pagetic bone showed an increased number of osteoclasts. These facts suggest a possible pathogenetic relationship between sarcoma and Paget's disease; the possibility of both processes having a viral aetiology is discussed.