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21/80. Alveolar rhabdomyosarcoma presenting as a peritonsillar abscess.

    A rhabdomyosarcoma of the head and neck region is a rare childhood neoplasm often presenting with vague symptoms that can easily mimic other diseases. We present an unusual case of an alveolar rhabdomyosarcoma of the soft palate in a three-year-old child, that presented as a peritonsillar abscess. ( info)

22/80. association of alveolar rhabdomyosarcoma with the beckwith-wiedemann syndrome.

    rhabdomyosarcoma (RMS) is a soft tissue tumor of childhood frequently diagnosed between the first and fifth year of life. Children with the beckwith-wiedemann syndrome (BWS), a congenital overgrowth syndrome characterized by exomphalos, macroglossia, and macrosomia, have an increased risk of developing childhood tumors including wilms tumor, hepatoblastoma, neuroblastoma, and RMS. Although an association between RMS and the BWS is well accepted, only four cases have been reported to date, and of these, three were reported as embryonal RMS. Based on these data, an association between BWS and embryonal RMS has been proposed. We report three additional cases of BWS with RMS and review the clinical data for each patient as well as the pathology of their tumors. All three cases of BWS had histology consistent with alveolar RMS and were diagnosed at 6 weeks and 5 and 13 years of age. In two of these BWS cases, constitutional defects of 11p15 imprinting were demonstrated. Furthermore, cytogenetic analysis of the tumors did not detect the t(2;13) or t(1;13) translocations that generate the PAX3- or PAX7-FKHR fusion proteins common to alveolar RMS. These observations suggest that the development of alveolar RMS tumors in BWS may occur without the chromosomal rearrangement producing the PAX-FKHR fusion protein. In summary, we present three new cases of RMS demonstrating a new association between BWS and an uncommon subtype of alveolar RMS. The absence of the translocations commonly associated with alveolar rhabdomyosarcoma suggests a common 11p15 pathway for alveolar RMS and BWS. ( info)

23/80. Primary cutaneous epidermotropic alveolar rhabdomyosarcoma with t(2;13) in an elderly woman: case report and review of the literature.

    We report a case of a primary cutaneous alveolar rhabdomyosarcoma presenting on the lower limb of a 60-year old woman. The tumor was characterized by aggregates of round blue cells in an alveolar growth pattern in the dermis and subcutis, with the additional unique finding of epidermotropism. By immunohistochemistry tumor cells were positive for vimentin, muscle-specific actin, desmin, myogenin, and Myo-D1 with focal positivity for CD56, neuron-specific enolase, and S-100 protein. Staining for pan-keratin, HMB-45, melan-A, epithelial membrane antigen, chromogranin, CD99, leukocyte common antigen, and alpha-smooth muscle actin was negative. interphase fluorescence in situ hybridization analysis from paraffin-embedded tumor demonstrated the presence of the translocation (2;13)(q35;q14) confirming the diagnosis. Further investigations revealed no tumor in the underlying deep soft tissues, and there was no evidence of metastasis in other organs. A local recurrence associated with a metastasis to a regional lymph node on the right groin was treated with an above-knee amputation and local radiotherapy to the groin area. The patient subsequently developed cutaneous metastases in the amputation stump and died 2 years after initial presentation. This case indicates that rhabdomyosarcoma may rarely present in the skin in adults and should be included in the differential diagnosis of primary cutaneous small round blue cell tumors not only in children but also in this age group. ( info)

24/80. Primary cutaneous alveolar rhabdomyosarcoma of the perineum.

    Primary alveolar rhabdomyosarcoma (RMS) involving perineal skin is extremely rare, particularly in the infant age group. We report a case of an alveolar RMS in a newborn with abnormal symmetrical perineal overgrowth, causing ambiguous morphologic structure of the genitalia. Clinical and imaging studies were suggestive of proteus syndrome with lymphatic malformation. Histologic examination of the mass showed cutaneous alveolar RMS with areas of embryonal and pleomorphic RMS features. Multiple superficially located, cystic-dilated spaces with loose edematous-mucoid hypocellular stroma gave a gross morphologic structure similar to that of lymphatic-type excrescences. ( info)

25/80. brain abscesses resulting from bacillus cereus and an aspergillus-like mold.

    An 11-year-old boy with alveolar rhabdomyosarcoma of the thigh experienced three instances of catheter-related bacteremia resulting from After two episodes of seizures, two low-density lesions in the right parietal lobe and the left corpus callosum with enhanced pericavitary opacity were detected. The catheter was removed. A brain biopsy sample grew and revealed dichotomously branched septate hyphae compatible with The patient was treated with ceftriaxone and liposomal amphotericin b for 12 and 52 weeks, respectively, until biopsy-confirmed resolution of the infections. ( info)

26/80. Congenital primary cutaneous rhabdomyosarcoma in a neonate.

    We report a case of congenital primary cutaneous rhabdomyosarcoma, solid alveolar type, presenting as a solitary skin lesion on the right upper lip of a 2-week-old infant boy. rhabdomyosarcoma originates from the embryonic mesenchyme precursor of striated muscle. Histologically it belongs to the group of "small round cell tumors." Its myogenic origin is ascertained by immunohistochemical studies positive for myogenin, muscle-specific actin, desmin, and myoglobin. Malignancy in the neonatal period is uncommon and the clinical management presents considerable challenges. Congenital alveolar rhabdomyosarcoma is a highly malignant tumor with no record of long-term survivors. Treatment options include chemotherapy, excision, and radiotherapy. This infant's tumor was responsive to chemotherapy and surgery and he was free of disease at the 6-month follow-up. ( info)

27/80. Primary perineal alveolar rhabdomyosarcoma metastatic to an extraocular muscle.

    A 17-year-old boy diagnosed with an alveolar rhabdomyosarcoma involving the perineum and with extensive lymphadenopathy was treated with chemotherapy yet developed metastases to the head and neck 6 months into therapy. Ten months after initial diagnosis, while receiving salvage chemotherapy and radiotherapy, he returned with pain on movement of his left eye, proptosis, and ptosis of the left upper eyelid. Computed tomography (CT) revealed a mass within the left lateral rectus muscle that biopsy confirmed to be metastatic alveolar rhabdomyosarcoma. Despite continued chemotherapy and radiotherapy, he ultimately died of the disease. Alveolar rhabdomyosarcoma from distant sites rarely metastasizes to the extraocular muscles. However, our case shows that alveolar rhabdomyosarcoma may metastasize to the orbit and involve a single muscle. ( info)

28/80. Primary and metastatic rhabdomyosarcoma in the breast: report of two pediatric cases.

    rhabdomyosarcoma (RMS) is a common childhood malignancy which can rarely be located in the breast. Here, we report two pediatric cases of breast RMS, one primary, the other secondary involvement. Primary one is alveolar, and the other embryonal subtype. Imaging findings with ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI) and a thorough review of literature are presented. ( info)

29/80. Alveolar rhabdomyosarcoma of the cervix.

    BACKGROUND: Alveolar rhabdomyosarcoma (RMS) has a high risk for local and distal failure. Multimodal management of a patient with alveolar RMS of the cervix uteri is outlined. CASE: A 39-year-old woman suffered from alveolar RMS of the cervix without involvement of uterus and parametrium. She was treated with total hysterectomy and left salpingo-oophorectomy. Systemic chemotherapy and pelvic irradiation were also offered. She remains in clinical remission 3 years after presentation. CONCLUSION: Postoperative chemotherapy and irradiation could be effective treatments for alveolar rhabdomyosarcoma of the cervix in adult patients. ( info)

30/80. Cytologic diagnosis of rhabdomyosarcoma in a child with a pleural effusion. A case report.

    BACKGROUND: A pleural effusion in children is usually caused by infectious diseases; malignant effusion is very uncommon. In a case of a malignant effusion in a child, a pleura-based metastasis of a neoplasm with a typically high prevalence in childhood has to be considered. Examples are neuroblastoma, nephroblastoma, Wilms' tumor, hepatoblastoma, malignant germ cell tumor and rhabdomyosarcoma. CASE: A 4-year old boy presented with a unilateral pleural effusion. Cytologic examination of the effusion revealed malignant small round cells admixed with very large cells with atypical nuclei. In formalin-fixed cell blocks prepared for immunocytochemistry the cells expressed desmin and myf-4. The cytologic diagnosis was consistent with metastatic rhabdomyosarcoma. Subsequent computed tomography revealed a mass above the diaphragm, which was biopsied. Histologic examination of a needle biopsy specimen confirmed the diagnosis of rhabdomyosarcoma. Molecular examination revealed a PAX3-FKHR fusion transcript specific to the alveolar type of rhabdomyosarcoma. CONCLUSION: This case illustrates the usefulness of immunocytochemistry on cell block preparations in diagnosing difficult cases of effusion cytology. ( info)
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