Adult diffuse hepatic hemangiomatosis: A case report and review of the literature

He, Song; Chen, Wenjing; Yang, Ying; Tang, Xiao-li; Zhou, Guangde; Jian-hui, Zhou; Wu, Chuanghong

doi:10.1016/j.clinre.2021.101789

Cited by 7 publications

(11 citation statements)

References 28 publications

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“…Hemangiomatosis is a vascular abnormality with an unknown etiology. Etiologic links have been reported between hemangiomatosis and inherited systemic diseases, such as Rendu–Osler–Weber disease and skeletal hemangiomatosis, and between hemangiomatosis and the use of medications, such as metoclopramide and estrogen ( 11 , 12 ). The correlation between hypothyroidism and diffuse hepatic hemagiomatosis has been described in infants but not in adults ( 13 ).…”

Section: Discussionmentioning

confidence: 99%

“…Ultimately, our patient received a combination regimen of prednisone, sirolimus, and thalidomide, which led to an improvement. Compared with KMS resulting from a single lesion or cutaneous hemangioma, the response to medications in diffuse hemangiomatosis with KMS is worse ( 12 ). The improvement of our patient’s platelet count and coagulation function did not reach the expected level after receiving combined therapy, which may be due to diffuse liver infiltration and extrahepatic lesions.…”

Section: Discussionmentioning

confidence: 99%

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Case Report: CTC1 mutations in a patient with diffuse hepatic and splenic hemangiomatosis complicated by Kasabach–Merritt syndrome

Guo

Niu

2023

Front. Oncol.

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Diffuse hemangiomatosis of the liver and spleen is rare. Currently, few studies are available on diffuse hepatic and splenic hemangiomatosis accompanied by Kasabach–Merritt syndrome (KMS). The conserved telomere maintenance component 1 (CTC1) gene contributes to telomere maintenance and replication by forming the telomeric capping complex. Herein, we report a case of diffuse hemangiomatosis in the liver and spleen accompanied by KMS in a 59-year-old woman who carried two novel heterozygous CTC1 variants: c.435+9A>C and c.3074C>T (p.Ala1025Val). Using next-generation sequencing, we detected mutations in the CTC1 gene in our patient, who had chief complaints of fatigue and abdominal distension complicated by severe thrombocytopenia and consumptive coagulopathy. Clinical symptoms, laboratory tests, and imaging findings led to the diagnosis of diffuse hepatic and splenic hemangiomatosis accompanied by KMS. The patient was treated with prednisone, thalidomide, and sirolimus, and her general condition was ameliorated at the 4-month follow-up with improved platelet count and coagulation function. A CTC1 gene mutation may be involved in the pathological process of vascular diseases. A combination treatment regimen of prednisone, thalidomide, and sirolimus may be effective for KMS.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Case Report: CTC1 mutations in a patient with diffuse hepatic and splenic hemangiomatosis complicated by Kasabach–Merritt syndrome

Guo

Niu

2023

Front. Oncol.

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“…10,14 Magnetic resonance imaging (MRI) is the most sensitive technique and shows lesions with hyperintensity on T2 weighted sequencing with progressive contrast enhancement. 4,6 The definitive diagnosis requires histopathological examination in doubtful and complicated cases. DHH can occur either in separate lobes or simultaneously in both hepatic lobes.…”

Section: Discussionmentioning

confidence: 99%

“…1,3 DHH is an unusual lesion with a pediatric preponderance. A PubMed search till May, 2022 along with the literature review by He et al 6 showed only 19 reported cases of adult DHH depicted in the English literature. 3,4,[6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] Approximately half of the DHH cases can be seen in association with GCH.…”

Section: Introductionmentioning

confidence: 99%

“…A PubMed search till May, 2022 along with the literature review by He et al 6 showed only 19 reported cases of adult DHH depicted in the English literature. 3,4,[6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] Approximately half of the DHH cases can be seen in association with GCH. 2 The etiopathogenesis of DHH is uncertain, although it has been diagnosed in association with hereditary disorders, such as skeletal hemangiomatosis and Osler-Weber-Rendu disease, and drugs like oral contraceptives (OC) and metoclopramide.…”

Section: Introductionmentioning

confidence: 99%

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Adult diffuse hepatic hemangiomatosis

Bhardwaj¹,

Parkhi²,

Kumar³

et al. 2022

Autops Case Rep

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Diffuse hepatic hemangiomatosis (DHH) is an uncommon vascular lesion, though hemangiomas are the commonest benign tumors of the liver. The etiology is largely unknown to date; however, its association with giant cavernous hemangiomas (GCH) has been reported in the literature. We present herein, the case of a 37-year-old hypothyroid woman with abdominal fullness for 2 months. The contrast-enhanced computed tomography revealed multiple wellencapsulated lesions involving the liver lobes and was diagnosed as giant cavernous hemangiomas. Most of them, except the deep-seated ones, were enucleated. Histopathological examination highlighted the presence of GCH with irregular margin, replacement of hepatic parenchyma, and presence of multiple micro-hemangiomas suggesting the possibility of DHH further substantiated by retrospective radiological assessment. No extrahepatic vascular lesion was noted, and the post-operative recovery and follow-up were uneventful. Adult DHH is an uncommon entity. The diagnosis of DHH and its distinction from GCH are important from the management and prognostic point of view as recurrence, extrahepatic manifestations, features of consumption coagulopathy, and death from the complications are not uncommon.

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A child with pancreatic hemangiomatosis presenting as obstructive jaundice: A rare case report

Wang

Kang

Hao

2022

Asian Journal of Surgery

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Adult diffuse hepatic hemangiomatosis: A case report and review of the literature

Cited by 7 publications

References 28 publications

Case Report: CTC1 mutations in a patient with diffuse hepatic and splenic hemangiomatosis complicated by Kasabach–Merritt syndrome

Case Report: CTC1 mutations in a patient with diffuse hepatic and splenic hemangiomatosis complicated by Kasabach–Merritt syndrome

Adult diffuse hepatic hemangiomatosis

A child with pancreatic hemangiomatosis presenting as obstructive jaundice: A rare case report

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