Pancreatic kaposiform hemangioendothelioma complicated by Kasabach–Merritt phenomenon: A rare entity

Mathew, Denny; Mahomed, Nasreen

doi:10.4102/sajr.v23i1.1760

Cited by 9 publications

(15 citation statements)

References 8 publications

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“…[ 14 ] KHE is a rare tumor known to occur in infants or young children, with an incidence of approximately 0.07/100,000 children per year. [ 15 ] The pancreas is an extremely rare location for a KHE in a child, with only nine reported cases between 1973 and 2015. [ 14 , 16 ] KHE is commonly associated with the Kasabach–Merritt phenomenon, [ 17 ] whereby a retrospective study of 107 patients with KHE between 1991 and 2009 reported that the phenomenon developed in 71% of the patients.…”

Section: Discussionmentioning

confidence: 99%

Malignant pancreatic tumor other than solid pseudopapillary tumor in pediatric patients

2021

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Pancreatic tumors, except solid pseudopapillary tumors (SPTs), are rare in pediatric patients. Herein, we report various types of pancreatic tumors in pediatric patients and review the literature regarding their treatments and prognosis. We retrospectively reviewed the data of pediatric patients who underwent surgery for pancreatic tumors, excluding SPTs, between January 2009 and December 2019 at Seoul National University Children's Hospital. A total of 35 pediatric patients were identified as having undergone surgery for pancreatic tumors. Of these patients, 30 were excluded because the tumor was identified as an SPT. The diagnoses of the five remaining (non-SPT) pancreatic tumors were pancreatic neuroendocrine tumor, mixed acinar neuroendocrine carcinoma, kaposiform hemangioendothelioma, and intraductal papillary mucinous neoplasm. All five patients survived; however, recurrence and liver metastasis were observed in one patient. The detailed demographics, treatments, and prognosis of each patient were reviewed. Despite the rarity and low i ncidence of pancreatic tumors in pediatric patients, four types of non-SPT tumors are reported here. Hence, the possibility of these should not be overlooked, especially since the diagnosis and adjuvant treatment differ vastly between the tumor types.

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Section: Discussionmentioning

confidence: 99%

Malignant pancreatic tumor other than solid pseudopapillary tumor in pediatric patients

2021

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“…It is associated with Kasabach-Merrit phenomenon, which is a combination of thrombocytopenia, hemolytic anemia, and consumptive coagulopathy (55). Histologically, KHE has an infiltrative growth pattern consisting of nodules and sheets of compact spindle cells with slitlike lumens, distinguishing these tumors from other pediatric vascular tumors (53,54). Immunohistochemical staining reveals positivity for lymphatic endothelial markers (D2-D40, ERG, CD31, and CD34) (53,55) and negativity for glucose transporter protein type 1, which is a marker of infantile hemangioma, another childhood vascular tumor (52,55).…”

Section: Kaposiform Hemangioendotheliomamentioning

confidence: 98%

“…Kaposiform hemangioendothelioma (KHE) is an aggressive infiltrative vascular tumor that commonly manifests during infancy or early childhood, and it most often affects the skin (51). KHE involving the pancreas is exceedingly rare, with fewer than 10 pediatric cases reported in the literature, all of which occurred in individuals within their first 3 years of life (52)(53)(54)(55).…”

Section: Kaposiform Hemangioendotheliomamentioning

confidence: 99%

“…KHE involving the pancreas can cause symptoms of obstructive jaundice and progressive abdominal distention (53,54). It is associated with Kasabach-Merrit phenomenon, which is a combination of thrombocytopenia, hemolytic anemia, and consumptive coagulopathy (55).…”

Section: Kaposiform Hemangioendotheliomamentioning

confidence: 99%

“…US may demonstrate a homogeneous soft-tissue mass (53). CT and MRI reveal an infiltrative soft-tissue lesion with ill-defined margins (52).…”

Section: Kaposiform Hemangioendotheliomamentioning

confidence: 99%

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Pancreatic Masses in Children and Young Adults: Multimodality Review with Pathologic Correlation

Qiu¹,

Trout²,

Ayyala³

et al. 2021

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Masses and masslike lesions of the pancreas are uncommon in the pediatric population. However, owing to overlapping clinical and imaging features, it can be challenging to differentiate the various causes of pediatric pancreatic masses at initial patient presentation. Clinical data such as patient age, signs and symptoms at presentation, laboratory test results, and potential underlying cancer predisposition syndrome can be helpful when formulating a differential diagnosis. US may be the first imaging study to depict a pancreatic mass in a child, as this examination is frequently performed in children with nonspecific abdominal signs and symptoms because of its wide availability and relatively low cost and the lack of a need for sedation or anesthesia. CT or MRI is typically required for more thorough characterization of the mass and surgical planning. Complete characterization of pancreatic masses includes assessment of vascular involvement, local invasion, and extrapancreatic spread of tumor. The authors provide an up-to-date comprehensive review of the clinical manifestations, histopathologic features, and imaging findings of primary and secondary tumors of the pancreas in children and young adults. Advances in imaging, current prognostic information, and treatment paradigms also are highlighted. Finally, nontumorous masslike lesions of the pediatric pancreas, including vascular malformations, cystic disorders (eg, von Hippel-Lindau syndrome, cystic fibrosis), intrapancreatic accessory spleen, and autoimmune pancreatitis, are discussed.

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Infantile Kaposiform hemangioendothelioma in a female patient complicated with severe obstructed jaundice: a case report

et al. 2022

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Background Kaposiform hemangioendothelioma (KHE) is a rare locally aggressive vascular neoplasm that occurs mainly in the pediatric population. KHE usually originates just underneath the skin and affects deeper tissues through infiltrative growth; however, visceral tissue involvement is quite rare. Case presentation An 8-month-old girl with jaundice and acholic stool was referred to our hospital for further evaluation of a hepatoduodenal ligament tumor. A blood examination revealed high bilirubin and liver enzyme levels, but no signs of coagulopathy. The first attempt at a diagnostic surgical procedure did not provide sufficient diagnostic information. However, the histopathological diagnosis of the cystic duct excised in the second surgery indicated KHE. Therefore, in our case, KHE was considered a cause of obstructive jaundice. Sirolimus (rapamycin) was initiated, and the patient was discharged 7 months after admission. Conclusions In cases of atypical hypervascular lesions in the abdominal cavity, especially in the pediatric population, it is important to consider the possibility of KHE, and surgical intervention with proper strategies is required for diagnosis, followed sequentially by promising treatments.

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Pancreatic kaposiform hemangioendothelioma complicated by Kasabach–Merritt phenomenon: A rare entity

Cited by 9 publications

References 8 publications

Malignant pancreatic tumor other than solid pseudopapillary tumor in pediatric patients

Malignant pancreatic tumor other than solid pseudopapillary tumor in pediatric patients

Pancreatic Masses in Children and Young Adults: Multimodality Review with Pathologic Correlation

Infantile Kaposiform hemangioendothelioma in a female patient complicated with severe obstructed jaundice: a case report

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