Primary small cell carcinoma of the liver, a rare entity

Otten, Martine; Sepehrkhouy, Shahrzad; Everdingen, Kaspar van; Haas, Lenneke E M

doi:10.1136/bcr-2013-201990

Cited by 6 publications

(1 citation statement)

References 3 publications

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“…Similar to other aggressive primary liver malignancies, PSCC present with hepatomegaly, upper abdominal discomfort or pain, fever, and jaundice in part of the patients (Sengoz et al 2003;Yuan et al 2006;Otten et al 2013). Serum AFP is, in most patients, not elevated, with the exception of the very uncommon PSCC with AFP secretion (Walshauser et al 2013).…”

Section: Clinical and Imaging Featuresmentioning

confidence: 99%

Rare Malignant and Semimalignant Epithelial Neoplasms of the Biliary Tract

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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The hepatobiliary tract is the site of an intriguing group of malignancies occurring in several other organs, extrapulmonary small cell carcinoma. In contrast to its bronchopulmonary counterpart, extrapulmonary small cell carcinomas occur with similar rates in female and male patients. With the exception of the gallbladder, the biliary tract is very rarely involved. Small cell carcinomas of the hepatobiliary tract belong to several categories, i.e., neuroendocrine type, mixed type, non-neuroendocrine type, and not otherwise specified types. Small cell carcinomas are highly aggressive neoplasms. Diagnosis and classification require immunohistochemical and molecular methods. Poorly differentiated or undifferentiated carcinomas of the bile ducts also include spindle cell carcinoma and carcinomas with rhabdoid features. Unusual neoplasms primary to the bile ducts also comprise hepatoid carcinoma and solid pseudopapillary tumor, a neoplasm better known from the pancreas.

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Section: Clinical and Imaging Featuresmentioning

confidence: 99%

Rare Malignant and Semimalignant Epithelial Neoplasms of the Biliary Tract

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

View full text Add to dashboard Cite

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Rare Malignant and Semimalignant Epithelial Neoplasms of the Biliary Tract

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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A case of malignant hyperlactaemic acidosis appearing upon treatment with the mono-carboxylase transporter 1 inhibitor AZD3965

et al. 2020

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A 47-year-old man with metastatic melanoma presented with refractory hyperlactaemic acidosis following the first dose of the mono-carboxylase transporter 1 inhibitor AZD3965 within a “first time in man” clinical trial. The mechanism of the agent and the temporal relationship suggested that this event was potentially drug related and recruitment was suspended. However, urinary metabolomics showed extensive abnormalities even prior to drug administration, leading to investigations for an underlying metabolic disorder. The lack of clinical symptoms from the elevated lactate and low blood glucose suggested a diagnosis of “hyper-Warburgism”, where the high tumour burden was associated with extensive glucose uptake and lactate efflux from malignant cells, and the subsequent impact on blood biochemistry. This was supported by an FDG-PET scan showing extensive glucose uptake in numerous metastases and lack of uptake in the brain. A review of the literature showed 16 case reports of “hyper-Warburgism” in non-haematological malignancies, none of them with melanoma, with most associated with a poor outcome. The patient was treated symptomatically, but died 2 months later. The development of AZD3965 continues with the exclusion of patients with elevated plasma lactate at screening added to the protocol as a safety measure.Trial identification number ClinicalTrials.Gov. NCT01791595.

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Primary small cell carcinoma of the liver, a rare entity

Cited by 6 publications

References 3 publications

Rare Malignant and Semimalignant Epithelial Neoplasms of the Biliary Tract

Rare Malignant and Semimalignant Epithelial Neoplasms of the Biliary Tract

Rare Malignant and Semimalignant Epithelial Neoplasms of the Biliary Tract

A case of malignant hyperlactaemic acidosis appearing upon treatment with the mono-carboxylase transporter 1 inhibitor AZD3965

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