Pleomorphic rhabdomyosarcoma metastasis to small intestine causing intussusception

Xi, Shiwen; Tong, Wei

doi:10.1097/md.0000000000013648

Cited by 4 publications

(3 citation statements)

References 8 publications

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“…The incidence of small bowel metastases is low, and misdiagnosis and missed diagnosis easily occur due to nonspecific symptoms. We reported the second case of small bowel intussusception caused by PPR metastasis, suggesting that the possibility of metastasis should be considered regardless of the rarity or site location [17]. Intussusception in adults should be investigated.…”

Section: Discussionmentioning

confidence: 99%

Small bowel metastasis from pulmonary rhabdomyosarcoma causing intussusception: a case report

Sun

Shen

2019

BMC Gastroenterol

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Background Rhabdomyosarcoma (RMS), especially primary pulmonary RMS, is an extremely rare type of soft tissue sarcoma in adults. Small bowel is an uncommon site for metastases. Case presentation This report described an unusual case of jejunum metastasis from primary pulmonary RMS causing intussusception in a 75-year-old man. The patient consulted for 2 weeks of continuous dyspnea. Chest computed tomography (CT) demonstrated a large mass involving the left lower lobe. Transthoracic biopsy confirmed the existence of pleomorphic RMS. Immunohistochemical studies showed positive findings about desmin and MyoD1. The results of gastroscopy, colonoscopy and abdominal CT were all negative. Positron emission tomography/CT demonstrated a fluorodeoxyglucose-reactive large lesion in the left lower lobe without metastatic lesions. The patient received synchronous chemoradiotherapy. After 9 months, the patient presented with intermittent upper abdominal pain with nausea and vomiting. CT showed small bowel dilatation secondary to intussusception. The patient subsequently received laparotomy, and the intussuscepted small bowel segment was resected. Histological examination revealed pleomorphic RMS involving the mucosa, submucosa, and muscular tissues. Conclusions RMS is highly aggressive and metastatic. The metastatic disease can rapidly progress to cause subsequent complications. The possibility of small bowel metastasis should be considered, although it is extremely rare.

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Section: Discussionmentioning

confidence: 99%

Small bowel metastasis from pulmonary rhabdomyosarcoma causing intussusception: a case report

Sun

Shen

2019

BMC Gastroenterol

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“…Because of aggressive growth in a limited anatomical space, cranial nerve paralysis may occur [121]. Rhabdomyosarcoma localized within the urogenital pathways, pelvis minor, or the abdominal cavity may give various symptoms such as chronic abdominal pain [122], bleeding from the birth canal [97], dysuria [123], jaundice [124], intussusception [125], or intestinal obstruction [126]. Edema, often painless, appearing in the case of RMS of the extremities or in the vicinity of the genital organs, may be ascribed to mechanical damage, which delays appropriate diagnosis.…”

Section: Clinical Picturementioning

confidence: 99%

Diagnosis and treatment of rhabdomyosarcomas

et al. 2023

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Rhabdomyosarcoma (RMS) is a soft tissue sarcoma. The primary tumor is most commonly localized in the head and neck, the urogenital system, or the limbs. Classification by the World Health Organization has distinguished four histopathological RMS subtypes: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing. Differential diagnosis of RMS includes melanoma, malignant neoplasm of peripheral nerve sheaths, liposarcoma, and PE-Coma. Among typical cytogenetic changes in RMS are chromosomal translocations t(2;13)(q35;q14) and t(1;13) (p36;q14). They lead to the formation of fusion genes that have a prognostic value. In the course of RMS, changes may also be present in signaling pathways, including RAS-PI3K, Wnt/b-catenin, receptor tyrosine kinase pathways, and myogenesis regulation. In 30% of patients at the time of diagnosis of RMS, distant metastases are present, most commonly to lungs, lymph nodes, bones, and bone marrow. Treatment of patients with RMS requires a multidisciplinary approach, and steadily perfected diagnostic techniques contribute to the individualization of therapeutic strategies. Optimal treatment of localized RMS is based on surgery combined with radiotherapy and chemotherapy. If distant metastases are present, the basic therapeutic method is multidrug chemotherapy, most frequently based on vincristine, dactinomycin, ifosfamide/cyclophosphamide, and etoposide. Despite intensive treatment, the 5-year survival index for RMS is not greater than 50%. There are still no unequivocal guidelines concerning the treatment in patients with local or distant recurrences.

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“…Pleomorphic RMS has been reported arising in the retroperitoneum, [25][26][27] adrenal, [28][29][30] kidney, 31,32 pancreas, 33 anterior abdominal wall, 27,34,35 omentum, 36 small intestine, 37,38 and liver 39 have been reported. Other studies reporting cases in the "trunk", "abdomen", "abdominal cavity" and "trunk wall" were noted although our search found only four cases specifically reported as pleomorphic RMS diagnosed in the abdominal wall (Table 1).…”

Section: Literature Review and Case Reportmentioning

confidence: 99%

Pleomorphic Rhabdomyosarcoma: A Systematic Review with Outcome Analysis and Report of a Rare Abdominal Wall Lesion

Deb

Suster

2022

Int J Surg Pathol

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Introduction. Pleomorphic rhabdomyosarcoma (RMS) is an aggressive and rare malignant neoplasm with a poor prognosis. As its name suggests, this tumor exhibits extensive pleomorphism with features of skeletal muscle differentiation. Due to its rarity, its diagnosis is often a clinical and pathological challenge. Since only small case series and a few scattered case reports exist in the literature, the impact of different demographic features, tumor site, and/or treatment modality on patient outcomes has yet to be extensively studied. Methods. We report a case of a pleomorphic RMS presenting atypically as an abdominal wall mass. We have also analyzed the National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) database to determine the factors affecting the outcome of this neoplasm. Moreover, we present a review and summary of pleomorphic RMS cases arising from the abdominal wall reported in the English language literature. Results. We found two hundred and forty-two cases of pleomorphic RMS in the SEER database. The majority of the patients were diagnosed after the age of 40, with the age of diagnosis showing a unimodal distribution. The majority of the patients were Caucasian (82%) and male (59%). Age of diagnosis, tumor stage, and surgical management significantly affected the patients’ outcome, while patients’ ethnicity, sex, or tumor site did not affect the outcome. We only found five previously reported cases of pleomorphic RMS arising from the abdominal wall. Conclusions. Pleomorphic RMS arising from the abdominal wall is extremely rare. Our data sheds light on the factors affecting the outcome of pleomorphic RMS. We have also discussed the challenges involving the histopathological diagnosis of this rare neoplasm and how to best approach this task.

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Pleomorphic rhabdomyosarcoma metastasis to small intestine causing intussusception

Cited by 4 publications

References 8 publications

Small bowel metastasis from pulmonary rhabdomyosarcoma causing intussusception: a case report

Small bowel metastasis from pulmonary rhabdomyosarcoma causing intussusception: a case report

Diagnosis and treatment of rhabdomyosarcomas

Pleomorphic Rhabdomyosarcoma: A Systematic Review with Outcome Analysis and Report of a Rare Abdominal Wall Lesion

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