Adult Kaposiform Hemangioendothelioma of the Leg Complicated by Kasabakh Meritt Phenomenon.

Ribag, Yassamina

doi:10.21474/ijar01/9392

Cited by 1 publication

(1 citation statement)

References 21 publications

(43 reference statements)

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“…KMP in children with KHE tend to be large, and because of intralesional platelet trapping, cause widespread thrombocytopenic manifestations. A cascade of consumption coagulopathy follows from the interaction of abnormally proliferating endothelium within the KHE and the platelets, secondarily activating the clotting cascade (7). It is inherently refractory to transfused platelets, thus is considered to be a potentially life-threatening disorder.…”

Section: Discussionmentioning

confidence: 99%

Kaposiform Hemangioendothelioma of the Sublingual Gland in an Adult: Resection is the Key.

Chowdhary

Sekar

Saxena

et al. 2021

Preprint

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Introduction: Kaposiform haemangioendotheliomas (KHE) are extremely rare, borderline malignant, locally aggressive vascular tumours. Although more commonly seen in childhood and adolescents, some very rare reports in adults do exist. KHE is usually found in the retroperitoneum, over the extremities and the soft tissues of the trunk, mediastinum and the head and neck regions. Prior published reports have shown a strong association with the Kasabach-Merritt phenomenon (KMP). We present a very rare case of KHE of the sublingual gland in an adult, which was not associated with KMP and was resected completely without any sequelae. Case report: A 42-year-old female presented with a painful swelling over the floor of the mouth on the right for a year. Clinical examination revealed a bi-digitally palpable, non-transilluminant lesion on the right side of the lingual frenulum. Radiological investigations suggested the possibility of a benign soft tissue tumour arising from the sublingual gland. The patient underwent an intraoral excision of the lesion. The biopsy revealed spindle-shaped tumour cells in fascicles, surrounded by vascular spaces, suggestive of a KHE. The patient was offered radiation therapy, which she declined and continued to be on follow-up for two years. She remains symptom-free to date.Conclusion: Excision of a KHE of the floor of the mouth proved to be sufficient, without the evidence of any residual lesion or progression of symptoms. To the best of our knowledge, our case represents the first reported case in the literature of a KHE arising from the salivary glands in an adult.

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Section: Discussionmentioning

confidence: 99%