Cases reported "Neoplasms, Second Primary"

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1/36. Uterine pleomorphic rhabdomyosarcoma in a patient receiving tamoxifen therapy.

    INTRODUCTION: tamoxifen has been used as adjuvant therapy for the treatment of breast cancer. Its use has been associated with the development of proliferative endometrial lesions such as polyps, hyperplasia, and carcinoma. Mesenchymal tumors including malignant mixed mullerian tumors, endometrial stromal sarcomas, adenosarcomas, and leiomyosarcomas have been more recently described with tamoxifen use. CASE REPORT: This report describes the first case of a pure uterine rhabdomyosarcoma in a patient receiving tamoxifen therapy. DISCUSSION: Although uterine rhabdomyosarcomas are rare tumors and may arise de novo, we discuss the possible role of tamoxifen in the development of these mesenchymal tumors.
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2/36. Rapid growth of a retroperitoneal rhabdomyosarcoma following right hemicolectomy for ascending colon cancer: report of a case.

    We report herein the case of a 69-year-old man in whom rapid growth of a retroperitoneal rhabdomyosarcoma occurred following hemicolectomy for ascending colon cancer. On his first admission for surgery, a small lesion, 1.5 cm in diameter, was detected adjacent to the inner side of the left kidney by abdominal axial computed tomography (CT), which was initially suspected to be a benign lesion; however, a postoperative follow-up CT scan done 5 months later revealed that the lesion had enlarged remarkably to 8 cm in diameter. Thus, total resection was performed under the presumed diagnosis of a malignant retroperitoneal tumor. The tumor was found to be adjacent to the inner portion of the left kidney and covered by Gerota's fascia. As it involved the ileolumbar muscle and had a metastatic lymph node, complete resection was performed. The resected specimen was 8.5 x 6.5 x 5 cm in size and was histologically confirmed as a retroperitoneal rhabdomyosarcoma of embryonal type. Two courses of adjuvant chemotherapy with adriamycin, vincristine, and cyclophosphamide were given, and the patient has shown no signs of recurrence for 2 years since his second operation.
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3/36. Colorectal adenocarcinoma as a second malignant neoplasm following rhabdomyosarcoma of the urinary bladder: a case report.

    Following improvements in therapy for childhood malignancies, the striking increase in survival rate over the past 30 years has led to the increase risk of developing second malignant neoplasms (SMNs). We report a case of colorectal carcinoma as a SMN, following treatment for rhabdomyosarcoma. The patient was diagnosed with rhabdomyosarcoma of the urinary bladder at his age of three years, and developed adenocarcinoma in the colon 13 years later. Histologic examination of the surgical specimen revealed adenocarcinoma involving the rectosigmoid area with radiation colitis in its background. The tumor cells showed strong immunoreactivity for p53 protein, suggesting the role of irradiation and p53 mutation in carcinogenesis. This case emphasizes the need for dose observation in survivors of early childhood malignancies treated with radiation and multiagent chemotherapy.
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4/36. Pleuropulmonary blastoma: an aggressive intrathoracic neoplasm of childhood.

    A case of a 4-year-old girl with pleuropulmonary blastoma is reported. Surgical resection of the tumor was performed and histologic examination revealed pleuropulmonary blastoma with rhabdomyosarcomatous differentiation. Postoperative chemotherapy was administered and 3 weeks after initiation of treatment protocol a second site of lesion in the retroperitoneum was revealed with extension to the mediastinum, which shared similar mesenchymal neoplastic characteristics to the previously diagnosed primary lesion. The girl died 4 1/2 months after initial evidence of disease because of brain metastasis, indicating a very aggressive neoplasm unresponsive to treatment.
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5/36. New techniques in hadrontherapy: intensity modulated proton beams.

    Inverse planning and intensity modulated (IM) X-ray beam treatment techniques can achieve significant improvements in dose distributions comparable to those obtained with forward planned proton beams. However, intensity modulation can also be applied to proton beams and further optimization in dose distribution can reasonably be expected. A comparative planning exercise between IM x-rays and IM proton beams was carried out on two different tumor cases: a pediatric rhabdomyosarcoma and a prostate cancer. Both IM x-rays and IM protons achieved equally homogenous coverage of the target volume in the two tumor sites. Predicted NTCPs were equally low for both treatment techniques. Nevertheless, a reduced low-to-medium dose to the organs at risk and a lesser integral non-target mean dose for IM protons in the two cases favored the use of IM proton beams.
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keywords = rhabdomyosarcoma
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6/36. rhabdomyosarcoma arising in a congenital melanocytic nevus.

    A variety of malignancies have been reported to arise within congenital melanocytic nevi, most commonly malignant melanoma, but rarely rhabdomyosarcoma, liposarcoma, and malignant peripheral nerve sheath tumor as well. There have been only three documented cases of rhabdomyosarcoma arising within congenital melanocytic nevi: two embryonal rhabdomyosarcomas and one mixed liposarcoma and rhabdomyosarcoma. One of these cases was also associated with neurocutaneous melanosis. We report a fourth case of rhabdomyosarcoma originating from a congenital melanocytic nevus. A 4-year-old girl presented with a large ulcerated nodule that developed within a hairy congenital nevus on her left gluteal and sacral regions. Her parents refused postoperative adjuvant therapy, and she died 13 months after surgical excision. Histologic sections showed a lesion with two distinct components. There was an expansile proliferation of pleomorphic cells within a fibromyxoid stroma. The neoplastic cells were spindled, and some had abundant eosinophilic globular cytoplasm with occasional cross-striations characteristic of rhabdomyoblasts. They strongly expressed desmin and myoglobin and were negative for S-100 protein and HMB-45. The tumor merged with an adjacent congenital melanocytic nevus characterized by a proliferation of uniform nonatypical melanocytes. The finding of both rhabdomyoblastic and melanocytic differentiation within the same lesion lends support to the hypothesis of their derivation from common pluripotential stem cells or neural crest cells.
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7/36. Potential reduction of the incidence of radiation-induced second cancers by using proton beams in the treatment of pediatric tumors.

    PURPOSE: To assess the potential influence of improved dose distribution with proton beams compared to conventional or intensity-modulated (IM) X-ray beams on the incidence of treatment-induced secondary cancers in pediatric oncology. methods AND MATERIALS: Two children, one with a parameningeal rhabdomyosarcoma (RMS) and a second with a medulloblastoma, were used as models for the purpose of this study. After defining the target and critical structures, treatment plans were calculated and optimized, four for the RMS case (conventional X-ray, IM x-rays, protons, and IM protons) and three for the irradiation of the spinal axis in medulloblastoma (conventional X-ray, IM x-rays, protons). Secondary cancer incidence was estimated using a model based on Publication No. 60 of the International Commission on Radiologic Protection. This model allowed estimation of absolute risks of secondary cancer for each treatment plan based on dose-volume distributions for the nontarget organs. RESULTS: Proton beams reduced the expected incidence of radiation-induced secondary cancers for the RMS patient by a factor of >or=2 and for the medulloblastoma case by a factor of 8 to 15 when compared with either IM or conventional X-ray plans. CONCLUSIONS: The potential for a significant reduction in secondary cancers with pediatric cancers after using proton beams (forward planned or IM) in the treatment of RMS and MBD in children and adolescents represents an additional argument supporting the development of proton therapy for most radiotherapy indications in pediatric oncology.
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8/36. rhabdomyosarcoma arising from a dormant dumbbell ganglioneuroma of the lumbar spine: a case report.

    STUDY DESIGN: A case report of a lumbar dumbbell tumor that showed a sequential change in histology from ganglioneuroma into rhabdomyosarcoma after long-term dormancy. OBJECTIVE: To present a case of non-neurogenic malignancy arising from ganglioneuroma. SUMMARY OF BACKGROUND DATA: Ganglioneuromas rarely form the dumbbell variety. Whereas conversion of ganglioneuroma into neurogenic malignancies occurs occasionally, between ganglioneuroma and mesenchymal malignancies it has so far been limited to a case in which ganglioneuroma developed after chemo- and radiotherapy for orbital rhabdomyosarcoma. methods: A 61-year-old man was referred with a recurrent retroperitoneal tumor that had been excised as ganglioneuroma. Using imaging, histologic, and immunohistochemical studies, a comparative analysis of the primary and recurrent tumors was performed. RESULTS: A total of 44 histologic sections were examined for the primary and the recurrent tumors that had been excised surgically, with an 8-month interval between resections. Histologic and immunohistochemical findings in the primary tumor were consistent with ganglioneuroma, whereas those in the recurrent tumor indicated rhabdomyosarcoma. CONCLUSIONS: It is assumed that the rhabdomyosarcoma arose from a dumbbell ganglioneuroma as a component of malignant ectomesenchymoma.
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keywords = rhabdomyosarcoma
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9/36. Orbital exenteration for secondary orbital tumors: a series of seven cases.

    BACKGROUND: Exenteration is indicated in patients with malignant neoplasms of orbital contents. It entails the removal of the eyeball together with its extraocular muscles and other soft tissues. Exenterations can be classified into (1) total, (2) subtotal, and (3) supertotal exenteration. Retrospectively study, we reviewed 7 patients that had received exenteration/subtotal exenteration with spontaneous granulation/myocutaneous flap implantation or eyelid-sparing exenteration with myocutaneous flap. Primary lesions, histopathological examination results, treatments, and recurrences are discussed. methods: A retrospective study of the years 1987 through 2000 disclosed 7 patients that underwent exenteration/subtotal exenteration. The patients ranged in age from 41 to 68 years. Two patients underwent total exenteration without socket augmentation; 4 patients underwent exenteration/ subtotal exenteration with immediate facial reconstruction, and 1 with delayed facial reconstruction. RESULTS: classification of the 7 patients showed that 2 had basal cell carcinoma of the skin, 2 had squamous cell carcinoma of the conjunctiva, 1 had squamous cell carcinoma of the paranasal sinus, 1 had rhabdomyosarcoma of the paranasal sinus, and 1 had intracranial meningioma. radiotherapy was performed in 6 of the patients and chemotherapy in 2. Central nerve system invasion was noted in 2 patients, and 1 died due to it. CONCLUSION: Secondary orbital tumors involved the orbit from adjacent tissues: paranasal sinuses, nasopharynx, lacrimal sac, conjunctiva, eyelid, intraocular tissue, and intracranial tissues. Combined surgeries are necessary for complete tumor removal. And the imaging studies should include the field of the orbit, sinus, and brain to search for the primary lesions.
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keywords = rhabdomyosarcoma
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10/36. Undifferentiated leiomyosarcoma showing various sarcomatous components with incidental B-cell lymphoma after tumor recurrence.

    We report a rare case of undifferentiated leiomyosarcoma (LMS) with incidental B-cell lymphoma in a 70-year-old woman. T2-weighted magnetic resonance images revealed a high signal intensity mass measuring 9 x 8 cm in the gluteus muscle. The pathological diagnosis of repeated surgery was undifferentiated LMS that included various sarcomatous components, such as fibrosarcomatous, rhabdomyosarcomatous, and malignant fibrous histiocytoma-like elements. A specimen from a supraclavicular lymph node showed the characteristics of malignant B-cell lymphoma (follicle type). Adjuvant chemotherapy or radiation therapy was not performed because of the patient's advanced age. The patient died from liver metastasis and dysfunction 5 years 8 months after the initial therapy. This is a rare case of LMS with malignant lymphoma. Considerable debate remains whether the B-cell lymphoma developed incidentally.
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