Cases reported "Neoplasms, Second Primary"

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1/16. Intraneural angiosarcoma and angiosarcoma arising in benign and malignant peripheral nerve sheath tumours: clinicopathological and immunohistochemical analysis of four cases.

    AIMS: Angiosarcomatous differentiation represents the least common form of heterologous differentiation in malignant peripheral nerve sheath tumours (MPNST), and is seen most frequently in patients with neurofibromatosis type 1. More rarely, it has been reported in patients without stigmata of neurofibromatosis, or in benign nerve sheath tumours and peripheral nerves. This study was undertaken to confirm this rare association. methods AND RESULTS: Four cases of angiosarcoma arising in a peripheral nerve, in a long-standing schwannoma and in two MPNST are described. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. An intraneural high-grade epithelioid angiosarcoma arose in the left posterior tibial nerve of a 78-year-old man, a well to moderately differentiated angiosarcoma was seen in an ancient schwannoma of the lateral neck in a 73-year-old women, and an angiosarcoma of varying grades of differentiation developed in a recurrent MPNST in the thigh of 38-year-old man. In addition a high-grade MPNST in the axillary region of a 30-year-old man showed foci of heterologous high-grade angiosarcomatous differentiation. The neural and endothelial lines of differentiation were confirmed in each case by positive immunohistochemical staining for neural and endothelial markers, respectively. In all cases tested, the neural differentiated cells stained immunohistochemically positive for antibodies against vascular endothelial growth factor. CONCLUSIONS: This study confirms the rare association of angiosarcoma arising in peripheral nerves, as well as in benign and malignant peripheral nerve sheath tumours.
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2/16. Hepatic angiomyolipoma in a 26-year-old Caucasian woman with a history of tibial osteosarcoma.

    We report on a 26-year-old Caucasian woman who was referred to the Department of Surgery complaining of general malaise, feeling of fullness with occasional vomiting and intermittent jaundice. The patient had previously suffered from tibial osteosarcoma of the left leg which was resected 13 years ago and subsequently treated with radiation and chemotherapy. During clinical investigations a 12 x 12 x 6.5 cm large mass was found in the left lobe of the liver. This was resected, and subsequently shown to be a sporadic hepatic angiomyolipoma. In order to investigate a possible link between the two tumours, we investigated mutations in the p53-gene, loss of heterozygosity (LOH) at p53, Rb and p16, c-Myc expression, and the telomerase activity of the angiomyolipoma and the osteosarcoma. Whilst the tibial osteosarcoma showed LOH at p16, no genetic alterations or increased telomerase activity were found in the angiomyolipoma. The occurrence of both these tumours in this patient is therefore probably a coincidence.
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keywords = tibia
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3/16. Treatment of solid tumors following allogeneic bone marrow transplantation.

    Second solid tumors are well known late complications after bone marrow transplantation. Treatment strategies are ill defined. We retrospectively evaluated treatment and outcome in a single institution. From August 1974 to July 1996, six solid tumors were observed in five of 387 patients 2 to 13 years after BMT, corresponding to a probability of developing a second solid tumor of 9% (1-17%, 95 CI) at 15 years: these comprised endometrial carcinoma, carcinoma of the thyroid gland, cervical carcinoma, sarcoma of the small intestine, osteosarcoma of the tibia and ovarian carcinoma. All five patients were treated as intensively as they would be without a history of BMT. At last follow-up four of the five patients were alive and without signs of tumor. We postulate that second solid tumors after BMT should be treated as de novo tumors. Early detection based on consequent clinical follow-up of the transplant patients might explain the relatively good outcome.
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4/16. Malignant transformation of a giant cell tumor 25 years after initial treatment.

    The current authors report a patient in whom a malignant fibrous histiocytoma developed long after a benign giant cell tumor of bone was removed from the same site. Twenty-five years after a benign giant cell tumor of the lateral condyle of the proximal tibia had been treated by curettage and iliac bone grafting without radiotherapy, a 53-year-old woman noted progressive pain and an enlarging mass in the same area. Radiographs showed osteolytic change, whereas magnetic resonance imaging indicated a tumor arising at the site of the giant cell tumor and extending beyond the bone. Examination of an open biopsy specimen showed a high-grade malignant fibrous histiocytoma with some areas rich in giant cells. After five courses of caffeine assisted intraarterial chemotherapy, the tumor was resected with an adequate margin, and the defect was reconstructed with an implanted prosthesis of corresponding shape. The extensor mechanism of the knee was reinforced using an allograft of fascia from the tensor fascia lata muscle. The resected specimen showed a good histologic response (95% tumor necrosis) to preoperative chemotherapy. Excellent function in the knee has been regained with no evidence of disease recurrence. caffeine potentiated chemotherapy was effective in minimizing the extent of tumor excision, in this case of high-grade malignant fibrous histiocytoma representing transformation from a benign giant cell tumor.
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keywords = tibia
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5/16. Metastatic osteosarcoma to the liver after treatment for synovial sarcoma: a case report.

    Metastatic osteosarcoma most commonly affects the lungs and other bones. Hepatic metastasis at the time of diagnosis is extremely rare. A 14-year-old boy with synovial sarcoma of the left popliteal fossa was treated with surgical resection, radiotherapy for microscopic residual disease, and 1 year of chemotherapy (vincristine, cyclophosphamide, dactinomycin, and doxorubicin). Approximately 10 years after the initial diagnosis, a secondary osteosarcoma developed in the left proximal tibia. Computed tomography at presentation showed bilateral pulmonary metastases and large ossified nodules in the liver that demonstrated abnormal avidity on 99mTc MDP bone scan indicating hepatic metastasis. Despite chemotherapy (cisplatin, ifosfamide, high-dose methotrexate, and dacarbazine), the patient died of progressive disease 4 months after the diagnosis of the second cancer. Hepatic metastasis was found at the time of diagnosis of a secondary osteosarcoma and manifested as ossified nodules. The risk of radiation-induced osteosarcoma should always be considered in decisions about treatment for soft-tissue sarcoma.
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6/16. Multifocal giant cell tumour of bone in a skeletally immature patient--a case report.

    giant cell tumor of bone is usually seen in adults affecting a single bone. Multiple giant cell tumour of bone occurring in skeletally immature patients is extremely rare. Multifocal giant cell tumor of bone in a ten year old boy involving upper end of humerus and tibia is being reported for its extreme paucity in literature.
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7/16. Multiple schwannomas of the foot. Case report and strategy for treatment.

    Determining the appropriate treatment of a benign tumor of a peripheral nerve in the foot and ankle region presents a clinical dilemma, as resection of the tumor will cause loss of nerve function and create the possibility of a painful neuroma. Several surgical solutions to this problem were used in the care of a patient who presented with painful bilateral Morton's neuromas and was found to have bilateral schwannomas on pathologic examination of the resected nerves. Subsequent evaluation for recurrent bilateral foot pain demonstrated multiple tumors along the tibial nerve in one foot. The patient also became aware of a painful mass on the dorsolateral aspect of one foot. review of the treatment options for this patient with multiple schwannomas provides a framework for decision making in the care of the patient with benign neural tumors of the foot.
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keywords = tibia
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8/16. Metachronous multicentric giant cell tumor: a case report and literature review.

    Metachronous multicentric giant cell tumors of bone are rare. The case of a 47-year-old woman who had a giant cell tumor of the ilium develop 24 years after resection of a giant cell tumor of the proximal tibia is reported. The initial and current surgical approaches for this patient are described. A literature review is presented to show that this patient had the longest disease-free interval documented for a patient with metachronous multicentric giant cell tumors.
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keywords = tibia
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9/16. Case report 714. Postirradiation osteosarcoma after radiation of metastatic skeletal lesion.

    Two cases of postirradiation osteosarcoma are presented--one in a 76-year-old woman with breast carcinoma and subsequent osteosarcoma after radiation therapy for a metastatic lesion in the right tibia, and the other in a 16-year-old girl with hepatocellular carcinoma metastatic to the left tibia and osteosarcoma after radiation therapy to that bone. Microscopically, both cases were high-grade spindle cell lesions with osteoid production. Both patients fared poorly. This is a rare complication of radiation therapy.
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keywords = tibia
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10/16. Simultaneous osteosarcoma lung metastasis and second primary lung cancer.

    Second malignant neoplasms (SMNs) after treatment of children and adolescent cancers have been extensively studied. lung cancer as an SMN is rare in all type of primary cancer in children and in osteosarcoma as well. The authors reviewed all cases of osteosarcomas treated at their institution from 1975 to 2000 and found 22 cases of SMN, only 1 involving lung cancer. The authors describe this latter case, a 17-year-old girl with a localized osteosarcoma of the tibia who developed simultaneously lung metastases of osteosarcoma and a primitive lung adenocarcinoma only 18 months from the diagnosis. lung cancer is unusual in youths as a primary malignancy or as a second primary malignancy. The occurrence of a primitive lung cancer together with lung metastases from osteosarcoma is even more unusual, and examination of all new lung nodules is highly recommended.
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