Kaposiform haemangioendothelioma of duodenum in a neonate

Kaposiform haemangioendothelioma, an endothelial borderline tumour, is typically seen in childhood involving extremities. It has been closely associated with a consumptive coagulopathy state, Kasabach-Merritt phenomenon (KMP). Extracutaneous involvement is uncommon. Intestinal involvement is quite u...

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Bibliographic Details
Published in:BMJ case reports 2021-02, Vol.14 (2), p.e239527
Main Authors: Khatri, Arti, Mahajan, Nidhi, Khan, Niyaz Ahmed, Dalal, Ashok
Format: Article
Language:English
Subjects:
Abdomen
Blood platelets
Case Report
Case reports
Diagnosis, Differential
Duodenal Neoplasms - diagnostic imaging
Duodenal Neoplasms - surgery
Duodenum - diagnostic imaging
Duodenum - surgery
Endoscopy
Hemangioendothelioma - diagnostic imaging
Hemangioendothelioma - surgery
Humans
Infant, Newborn
Intestinal obstruction
Kasabach-Merritt Syndrome - diagnostic imaging
Kasabach-Merritt Syndrome - surgery
Microscopy
Newborn babies
Patients
Pediatrics
Radiography - methods
Sarcoma, Kaposi - diagnostic imaging
Sarcoma, Kaposi - surgery
Thrombocytopenia
Vomiting
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Summary:Kaposiform haemangioendothelioma, an endothelial borderline tumour, is typically seen in childhood involving extremities. It has been closely associated with a consumptive coagulopathy state, Kasabach-Merritt phenomenon (KMP). Extracutaneous involvement is uncommon. Intestinal involvement is quite uncommon and can masquerade as an acute abdomen. A 24-day-old neonate presented with bilious vomiting and fever for 5 days. Sections from the resected gangrenous duodenum contained a submucosal tumour composed of infiltrating nodules of slit-like or crescentic CD34-positive spindled-to-flattened endothelial-lined vascular spaces along with zones of fibrosis. No nuclear pleomorphism or necrosis identified. The findings were classic example of kaposiform haemangioendothelioma with an absence of any deranged coagulation profile. The index case raises interest given its congenital incidental presentation at an uncommon site, like duodenum, and absence of coexistent KMP.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2020-239527