Primary embryonal botryoid-type rhabdomyosarcoma of the liver

Tutar, Nihal Uslu; Cevik, Belma; Ötgün, İbrahim; Tarhan, Nefise Çağla; Özen, Özlem; Çoşkun, Mehmet

doi:10.1016/j.ejrex.2006.10.005

Cited by 8 publications

(8 citation statements)

References 5 publications

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“…RMS is common in childhood and constitutes around 50% of all soft-tissue sarcomas. It constitutes <1% of all malignancies and 3% of all soft-tissue sarcomas in adults 3. It is most common at ages 2–6 and 15–18 years.…”

Section: Discussionmentioning

confidence: 99%

“…It is most common at ages 2–6 and 15–18 years. Head, neck and genitourinary organs are the most common sites and embryonal RMS is most common subtype of RMS in younger age group 2 3. Alveolar is the most common subtype in patients more than 10 years of age and pleomorphic RMS is a disease of the elderly 8.…”

Section: Discussionmentioning

confidence: 99%

“…Its occurrence in adults is rare. Most common sites of RMSs are head and neck, genitourinary organs, retroperitoneum and extremities 2 3. RMS in the liver is very rare.…”

Section: Introductionmentioning

confidence: 99%

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Primary embryonal rhabdomyosarcoma of the liver

2016

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Rhabdomyosarcomas are malignant neoplasms with striated muscle differentiation. This is the most common type of soft-tissue sarcoma in children, but occurs rarely in adults. Its occurrence in liver is infrequent. We report a case of primary hepatic embryonal rhabdomyosarcoma in a 67-year-old man. The tumour was occupying the left lobe of the liver with large component of lesion seen bulging in left subhepatic space indenting over the stomach, compressing the pancreas and gall bladder. A percutaneous biopsy was performed which revealed embryonal rhabdomyosarcoma. He underwent a successful left lobectomy with complete resection of the tumour, followed by adjuvant chemotherapy. The patient is free of disease at 24 months of follow-up. Hence, an early diagnosis, prompt surgical resection with negative resection margin along with adjuvant chemotherapy can provide complete remission. This case is extremely rare both due to the age of the patient at presentation and location of the tumour.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Primary embryonal rhabdomyosarcoma of the liver

2016

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“…Botryoid RMS is actually a subtype of embryonal RMS [14]. Embryonal RMS is the most frequent type of RMS in young children, alveolar RMS is the most frequent type in patients older than 10 years, and pleomorphic RMS is the most frequent type in advanced-age adults [3,13]. Among the adult patients in whom RMS originated in the liver, four had embryonal RMS and three had pleomorphic RMS.…”

Section: Case Presentationmentioning

confidence: 99%

“…RMS is the most common type of childhood soft tissue sarcoma, constituting 5 to 10% of all solid tumors in childhood. However, it rarely occurs in adults; soft tissue sarcomas account for less than 1% of all cancers in adults [1][2][3]. Although this tumor may occur anywhere in the body, it is uncommon in the liver.…”

Section: Introductionmentioning

confidence: 99%

Pleomorphic rhabdomyosarcoma of the liver in an adult: a rare case report

et al. 2020

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Background: Rhabdomyosarcoma (RMS), a malignant neoplasm that normally differentiates to form striated muscle, is the most common type of childhood soft tissue sarcoma. However, it infrequently occurs in adults and is uncommon in the liver. We herein report a case of RMS of the liver in an adult. Case presentation: A 73-year-old woman was admitted to our institution for investigation of a hepatic mass. She had been followed for primary biliary cirrhosis for the past 20 years. A contrast-enhanced computed tomography scan of the abdomen showed a 12-× 10-cm heterogeneous low-density mass lesion containing cystic and solid components. A percutaneous liver biopsy was performed, and poorly differentiated cancer containing an RMS celllike component was observed. The patient was diagnosed with RMS of the liver, and open surgery with right hepatic lobectomy was performed. Histopathological examination confirmed a diagnosis of pleomorphic RMS of the liver. The patient died of rapid progression of the tumor 6 months after the operation. Conclusions: The tumor site in the present case is rare. The details of this case add to the current evidence base regarding establishment of the standard diagnosis and treatment of this rare condition. We recommend consideration of RMS as a differential diagnosis for hepatic tumors.

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