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11/80. Blueberry muffin rash as a presentation of alveolar cell rhabdomyosarcoma in a neonate.

    Soft tissue sarcomas of childhood continue to present problems with pathologic diagnosis, staging and treatment. rhabdomyosarcoma, the most common soft tissue sarcoma, represents 4-8% of all malignant solid tumours in children. We report a case of congenital alveolar rhabdomyosarcoma who presented with "blueberry muffin"-like rash. A full-term female infant was noted at birth to have multiple skin lesions resembling blueberry muffin rash and an abdominal mass in the left iliac fossa, which appeared to be fixed to the posterior abdominal wall. There was no enlargement of liver and spleen, but her para-aortic lymph nodes were enlarged. biopsy from the mass confirmed the diagnosis of alveolar cell rhabdomyosarcoma. Molecular investigation for the t (2:13) translocation was negative. The infant received chemotherapy but died within 1 mo of diagnosis. ( info)

12/80. Congenital alveolar rhabdomyosarcoma: clinical and molecular distinction from alveolar rhabdomyosarcoma in older children.

    BACKGROUND: Congenital alveolar rhabdomyosarcoma (RMS) is extremely rare and invariably fatal with current therapy. Its clinical presentation is unusual and is distinct from RMS in older children, with > 50% of patients presenting with multiple cutaneous metastases. To the authors' knowledge, the biology of this condition has not been studied to date. methods: The clinical and pathologic findings of four cases of congenital RMS are presented along with those of other cases found in the literature. Molecular analysis was undertaken to detect the characteristic chromosomal translocations of alveolar RMS in three cases with suitable material. RESULTS: To the authors' knowledge, there are no recorded survivors of congenital alveolar RMS. Clinical and histopathologic findings in four patients with congenital alveolar RMS are detailed along with treatment responses. It now is recognized that tumor specific translocations can be detected in the majority of cases of alveolar RMS using the reverse transcriptase-polymerase chain reaction (RT-PCR) method. However, detailed molecular analysis using RT-PCR was unable to detect the presence of either the classic t(2;13) or variant t(1;13) translocation in three cases analyzed at the molecular level. CONCLUSIONS: To the authors' knowledge the optimal treatment for this rare tumor is not known. The longest survivor in the current series underwent myeloablative therapy with peripheral stem cell support, suggesting that more intensive treatment may be of value in this rare condition. None of the three tumors analyzed by RT-PCR harbored characteristic RMS translocations. The molecular biology of this highly malignant and aggressive congenital tumor appears to be different from alveolar RMS in older children and warrants further investigation. ( info)

13/80. Membrane-bound paracrystalloid structures in alveolar rhabdomyosarcoma.

    We report here a case of an alveolar rhabdomyosarcoma in a 25-year-old woman. Ultrastructural examination revealed a few periodical membrane-bound crystalloids, similar to those regarded as specific for alveolar soft-part sarcoma. Our study provides evidence supporting a myogenic origin of those periodic structures, and simultaneously a myogenic origin of alveolar soft-part sarcoma. ( info)

14/80. Solid variant of alveolar rhabdomyosarcoma with unbalanced t(2;13) and hypotetraploidy, without MYCN amplification.

    The histological subtype of alveolar rhabdomyosarcoma (AR) is characterised by the cytogenetic translocation t(2;13)(q35;q14) in approximately 70% of cases, a rearrangement rarely present in the embryonal rhabdomyosarcoma (ER) subtype. The MYCN gene is amplified in some cases of AR. We present a young man with an unusual pattern, namely solid variant of AR with hypotetraploidy and the t(2;13) in an unbalanced form. The MYCN gene was not amplified on FISH, but showed increased copy number, consistent with ploidy. ( info)

15/80. Fine-needle aspiration biopsy of alveolar rhabdomyosarcoma of the parotid: a case report and review of the literature.

    The cytologic features of an alveolar rhabdomyosarcoma arising within the parotid gland are described. The occurrence of this neoplasm as a primary malignancy in the parotid gland is very rare, and the tumor is usually not included in the cytologic differential diagnosis of parotid tumors. The diagnosis of the current case was achieved by a combination of fine-needle aspiration biopsy and frozen sections. Realizing the difficulty of recognizing this tumor both histologically and cytologically, we present this case to increase the awareness of this tumor's occurrence within the parotid gland and other salivary glands, and to highlight cytomorphologic features that will aid the pathologist in making the correct diagnosis. ( info)

16/80. Integrated diagnostic imaging of primary thoracic rhabdomyosarcoma.

    We report a rare case of primary thoracic rhabdomyosarcoma in a girl who was referred with acute chest pain, hacking cough, and wheezing. A chest X-ray revealed a complete opacity of the right hemithorax. Ultrasound revealed a right-sided pleural effusion and a solid mass above the liver dome, suggesting a neoplastic disease, which quickly led to further specific examination. Use of CT and MRI together with bone scintigraphy completed the investigation. The biopsy specimen showed a pattern of alveolar rhabdomyosarcoma. This case was reported to emphasize the role of US in the evaluation of a child with hemithorax opacity. ( info)

17/80. rhabdomyosarcoma in children: report of two cases.

    rhabdomyosarcoma of soft tissue is the commonest sarcoma in pediatric medicine and comprises 15% of the solid tumors treated in pediatric oncology. It has an ample variety of clinical presentations in diverse organs and also presents a gamut of histopathological patterns, which sometimes make diagnosis difficult. We report two cases of rhabdomyosarcoma: one of the embryonic histological variety and the second one of the alveolar variety, which presented themselves in the preauricular and masseteric areas respectively. Both patients were male, diagnosed and treated. ( info)

18/80. Alveolar rhabdomyosarcoma of the parotid gland.

    Alveolar rhabdomyosarcoma of the parotid gland is a rare condition. The treatment should involve an aggressive multi-modality approach incorporating surgical removal of the tumour, radiotherapy, and multi-agent chemotherapy. The presentation of this condition along with the most appropriate line of treatment is discussed. ( info)

19/80. Neonatal alveolar rhabdomyosarcoma with skin and brain metastases.

    BACKGROUND: Approximately 5-10% of patients with rhabdomyosarcomas (RMS) are diagnosed during the first year of life, and their clinical characteristics have been well documented. However, because RMS rarely occurs during the neonatal period, little is known about neonatal RMS. methods: Four patients with neonatal RMS were treated at St. Jude Children's research Hospital between 1962 and 1999. The authors report the results of a review of these patients and of cases described in the literature. Clinical, radiologic, and pathologic features of these patients and their outcomes were evaluated. RESULTS: One patient with embryonal RMS was treated successfully with a combination of systemic chemotherapy and local control measures. The other three patients had alveolar RMS. Two of them had multiple skin and subcutaneous metastatic nodules at the time of diagnosis and developed brain metastases early in their course. In one of these patients, the PAX3-FKHR fusion transcript was detected. Three other similar cases of neonatal alveolar RMS with metastases to the skin and brain have been reported in the literature. CONCLUSIONS: A distinct syndrome of neonatal RMS is described. This syndrome is characterized by alveolar histology, multiple skin and subcutaneous metastases, and fatal outcome as the result of early brain metastasis. ( info)

20/80. Tc-99m MDP, thallium-201 chloride and Tc-99m MAG3 renal uptake in subacute and chronic radiation nephritis compared.

    The authors present a comparison of the findings for thallium-201 (Tl-201), Tc-99m MAG3 and Tc-99m MDP in subacute and chronic radiation nephritis in a 9-yr-old boy who was treated by radiation therapy for alveolar rhabdomyosarcoma of the left chest wall by a radiation port that partially included the left kidney. Tl-201 imaging three and six months later showed a cortical defect in the left kidney due to radiation nephritis. Tc-99m MDP scan showed increased uptake on both occasions, but more marked in the subacute period than in the chronic period. Tc-99m MAG3 showed decreased concentration and increased cortical retention three months later. Six months after the radiation therapy, a cortical defect corresponding to the cortical area that showed increased parenchymal retention was more prominent in the Tc-99m MAG3 scan. In the present case, Tc-99m MDP, Tl-201 and Tc-99m MAG3 findings may provide useful information for understanding pathophysiological damage in the kidney after radiation. ( info)
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