Intraabdominal desmoplastic small round cell tumor: results of ifosfamide-based chemotherapy.

Intraabdominal desmoplastic small round cell tumor (IDSRT) is a rare neoplasm, which is malignant and typically occurs in young adults. Although IDSRT is chemotherapy-sensitive, most cases do not achieve a complete response (CR). Even if some cases achieve a CR, they often relapse and result in poor prognosis. We report two cases of IDSRT, in a 24-year-old man and a 23-year-old man, who were treated with ifosfamide-based chemotherapy. Case 1, a 24-year-old man, presented with an abdominal mass, and the diagnosis of the biopsy specimen at laparoscopy was IDSRT. He received 7 courses of ifosfamide-based chemotherapy, which obtained a partial response (PR). He had no surgical resection, except for the biopsy procedure, and he underwent a total of 21 courses of chemotherapy. He died 36 months after diagnosis because of tumor regrowth and peritoneal bleeding. Case 2, a 23-year-old man, whose biopsy material allowed a diagnosis of IDSRT, was treated with 18 courses of chemotherapy, using ifosfamide, that resulted in a PR. This was followed by resection of the abdominal lesions, and he received an additional 2 courses of adjuvant chemotherapy. Four months after, completion of these 2 courses, the tumor recurred, and he died of diffuse pulmonary metastasis 36 months after diagnosis. This case report examines the results of ifosfamide-based chemotherapy in our two patients.

Clear cell sarcoma arising from the chest wall: a case report.

Clear cell sarcoma is a rare malignant soft tissue neoplasm that usually arises adjacent to tendons or aponeuroses. The clinical course is rather slow, with repeated local recurrences followed by late metastases and eventual death. The principal sites of this neoplasm are the extremities, but tumors do occur in the trunk on rare occasions. We report a case of clear cell sarcoma arising from the chest wall. The patient, a 20-year-old woman, had noticed a chest wall mass and pain for 2 years. Biopsy of the mass showed abundant nests of round cells with clear cytoplasm. On immunohistochemical examination, tumor cells were strongly immunoreactive for S-100 and HMB-45. A diagnosis of clear cell sarcoma was confirmed. There was no other lesion found in the patient through routine imaging studies. She was treated with two courses of chemotherapy using ifosfamide, carboplatin, and etoposide. Subsequently, the tumor, including adjacent tissue, the chest wall, and sternum, was resected with a wide margin; and the defect of the chest wall was covered with Marlex mesh fabric, regin, and a musculocutaneous flap. She has shown no symptoms or signs of recurrence during 2 years of follow-up.

Periosteal osteosarcoma with secondary bone marrow involvement: a case report.

Periosteal osteosarcoma is an exceedingly rare type of chondroblastic osteosarcoma, showing a rather good prognosis, and secondary bone marrow involvement is unusual. However, there have been some reports describing periosteal sarcoma involving medullary bone. We encountered a patient, a 38-year-old man, who had a bone surface tumor in the left tibia. An X-ray showed an erosive cortical mass extraosseous portion, located in the diaphysis of the tibia. Other images revealed a thin cortex, periosteal reactions, coarse mineralization in the extraosseous portion, and bone marrow involvement. Grossly, surgical materials showed that the tumor mainly existed at the periosteal portion, only a part of the cortex was destroyed, and there was medullary involvement throughout. Histological examinations showed a predominantly chondroid component with malignant osteoid formation. On the basis of the histological macroscopic and microscopic findings, we made the diagnosis of periosteal osteosarcoma with secondary bone marrow involvement.

Metastatic Bednar tumor (pigmented dermatofibrosarcoma protuberans) with fibrosarcomatous change: a case report.

Bednar tumor (pigmented dermatofibrosarcoma protuberans) is a variant of dermatofibrosarcoma protuberans (DFSP) that constitutes 5% of all DFSP and has a very low rate of distant metastases. We encountered a rare case of Bednar tumor with multiple different distant metastases. A 51-year-old man, who had had a history of mass resection in his left shoulder 4 years previously, was referred to our institution, complaining of a recurrence of the shoulder mass. The histological diagnosis of primary tumor was Bednar tumor, and he underwent resectional surgery for the recurrent lesion. A second local relapse, lung metastasis, retroperitoneal metastasis, and metastasis to the sigmoid colon have occurred. The recurrence lesion and all metastatic lesions were resected surgically. The histological features of all specimens showed fibrosarcomatous change. Seven months after the last surgical resection, the tumor recurred and the patient died of multiple abdominal metastases 10 years after the first surgical treatment.

Fracture-dislocation of the hip with ipsilateral femoral neck fracture.

Fracture-dislocation of the hip associated with fracture of the femoral neck and intrapelvic intrusion of the femoral head is a rare injury. In this case we performed open reduction for the femoral neck fracture, and external fixation for the pelvic fracture by Judet-Meyers method. As a result the patient has not complained of pain, although he developed arthrokleisis due to ectopic ossification around the femoral head, as well as femoral head necrosis.