Neoadjuvant Everolimus for Adult Giant Mesenteric Cystic Lymphangioma with mTOR Pathway Activation

Gruel, Nadège; Bonvalot, Sylvie; Mir, Olivier; Watson, Sarah

doi:10.1002/onco.13775

Cited by 3 publications

(1 citation statement)

References 7 publications

(10 reference statements)

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“…Based on the size of the dilated vessels, lymphangiomas are classified in capillary, cavernous and cystic lymphangiomas [ 4 , 21 ]. If capillary and cavernous lymphangiomas are mainly developed in the submucosa of the GI tract, cystic lymphangioma is rather a mesenteric lesion developed as result of the mTOR pathway activation, which is therapeutically inhibited by everolimus [ 21 , 22 ].…”

Section: Vascular Hamartomasmentioning

confidence: 99%

Nonpolypous Hamartomas of the Gastrointestinal Tract: An Updated Review on Classification, Denominations, and Clinical Management

Gurzu

Jung

2022

Journal of Oncology

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Purpose. To perform the first systematic report about histological subtypes of nonpolypous hamartomas of the gastrointestinal (GI) tract, from esophagus to anal canal. Design. From over 19,000 studies about hamartomas, most of them published as case series or case presentations, we have selected the most representative ones for the GI tract, excluding polyposis syndromes. To have a whole picture of these hamartomas, all of the data were combined with the personal experience of the authors who are GI pathologists. Results. The examined articles showed predominance of vascular and combined vascular and mesenchymal hamartomas. Arteriovenous hamartomas or Brunner gland hamartomas are mainly diagnosed in the small intestine, with preponderance for duodenum. Other malformations such cavernous hamartomas are more specific for the colorectal segments, whereas chondromatous hamartomas or those derived from the neural ectoderm were mostly reported in the esophagus. As newly recognized entities were admitted in the last years, misdiagnosis is frequent, and the best therapeutic approach is far to be known. Conclusion. Even rare, hamartomas of the GI tract need to be differentiated from tumors and familial polyposis syndromes. Knowing their proper denominations and possible complications is valuable for gastroenterologists, pathologists, and surgeons, to be aware in the differential diagnosis.

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Section: Vascular Hamartomasmentioning

confidence: 99%

Nonpolypous Hamartomas of the Gastrointestinal Tract: An Updated Review on Classification, Denominations, and Clinical Management

Gurzu

Jung

2022

Journal of Oncology

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Clinical characteristics and risk factors for acute abdomen in patients with abdominal lymphatic malformations

Yang,

Qiu,

Yang

et al. 2024

Journal of Vascular Surgery: Venous and Lymphatic Disorders

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Intra-Abdominal Multi Cystic Lymphangiomas: A Case Series with Adult and Pediatric Literature Review

Avani,

Pramath,

Dan

et al. 2024

Arch Clin Gastroenterol

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Background: Multi-cystic Lymphangioma (MCL) is a rare intra-abdominal entity in adults. The diagnosis of a core biopsy specimen is challenging due to its rarity in general surgical pathology service. Invasive surgical management can be avoided with accurate core biopsy diagnosis. Methods: We present six cases of adult intra-abdominal MCL and highlight their clinical, radiological, and pathological characteristics. Demographic, clinical, radiological, and histopathological parameters were collated for analysis. Results: Six patients (out of 958,574 cases) were identified (prevalence: 0.6/100,000 cases). Previous abdominal surgery (67%, n = 4) and abdominal pain with palpable abdominal mass (67%, n = 4) at presentation were notable in this cohort. Abdominal CT showed cystic mass with septations, punctate calcification, and fatty halos raising the differential diagnosis of mesenteric cyst (n = 2), duplication cyst (n = 2), or disseminated metastases (n = 2). MCL was distributed across the jejunal mesentery (n = 3) and was managed with exploratory laparotomy and resection (n = 4). Characteristic histopathological features include multiple variably sized cystic spaces lined by attenuated flattened epithelium, cysts filled with proteinaceous fluid, and interspersed by stroma with several dense lymphoid aggregates. The lymphatic endothelium showed positivity for D2-40 and CD31. Post-operative follow-up at 50.4 ± 49.2 months did not show any clinical or radiological recurrence. Conclusion: Adult intra-abdominal MCL are rare and radiologically indistinguishable from other intra-abdominal lesions. Diagnostic uncertainty on core biopsy evaluation in our series required invasive surgical exploration. The recognition of the histological triad of lymphoendothelial cysts, smooth-muscle or fibrous stroma, and associated lymphoid aggregates are diagnostic for MCL. Cystectomy alone is curative (144 months without recurrent).

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Neoadjuvant Everolimus for Adult Giant Mesenteric Cystic Lymphangioma with mTOR Pathway Activation

Cited by 3 publications

References 7 publications

Nonpolypous Hamartomas of the Gastrointestinal Tract: An Updated Review on Classification, Denominations, and Clinical Management

Nonpolypous Hamartomas of the Gastrointestinal Tract: An Updated Review on Classification, Denominations, and Clinical Management

Clinical characteristics and risk factors for acute abdomen in patients with abdominal lymphatic malformations

Intra-Abdominal Multi Cystic Lymphangiomas: A Case Series with Adult and Pediatric Literature Review

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