An extrapulmonary manifestation of lymphangioleiomyomatosis: A rare case report

Tümay, Latif Volkan; Güner, Osman Serhat; Zorluoğlu, Abdullah

doi:10.1016/j.ijscr.2017.10.057

Cited by 5 publications

(6 citation statements)

References 9 publications

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“…Microscopically detectable smooth muscle cell proliferation is the main cause of bronchiole obstruction and alveolar air blockage leading to spontaneous pneumothorax. In addition, blockage of the lymphatic vessels leads to chylothorax and chylic ascites [23][24][25]. Research on extrapulmonary LAM treatment has been limited [16][17][18][19].…”

Section: Discussionmentioning

confidence: 99%

Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study

Marciniak,

Nawrocka-Rutkowska,

Brodowska

et al. 2023

JPM

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Lymphangioleiomyomatosis (LAM) is characterized by lung cysts that cause lung deterioration, changes in the lymphatic system, and tumors in the kidneys. It mainly affects women of reproductive age and is a progressive disease. LAM can occur as an isolated disease or coexist with tuberous sclerosis (TSC). The source of LAM cells is unknown. Patients with confirmed LAM should be treated with an mTOR inhibitor, sirolimus, or everolimus. We present a case of LAM with TSC in a patient whose symptoms, including those in the lymph nodes and chyaloperitoneum, mainly concern the abdominal cavity.

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Section: Discussionmentioning

confidence: 99%

Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study

Marciniak,

Nawrocka-Rutkowska,

Brodowska

et al. 2023

JPM

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“…In patients with extrapulmonary LAM (E-LAM), mediastinal and upper abdominal retroperitoneal lymphadenopathies (LAPs), and renal angiomyolipomas are common. [ 15 ] A lymphangioleiomyoma is a cystic mass found in the lymphatic system. The signal intensities of the center area on T1-weighted imaging and T2-weighted imaging are homogeneous and similar to those of ascites.…”

Section: Discussionmentioning

confidence: 99%

Primary cystic and solid neuroendocrine tumor of the retroperitoneum

et al. 2021

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Rationale: Primary neuroendocrine tumors (NETs) of the retroperitoneum are extremely rare. The purpose of this case report is to highlight the unusual growth pattern and radiologic features of primary retroperitoneal NETs. Patient concerns: A 46-year-old woman was found to have a retroperitoneal cystic and solid mass during a physical checkup. Diagnoses: The mass was mainly multiseptated in the cystic portion and had a bead-like, lobulated appearance. The solid portion showed restricted diffusion on diffusion-weighted imaging and obvious homogeneous enhancement. The cystic portion showed ring-like and septal enhancement. The patient was diagnosed with a grade 2 (G2) NET of the retroperitoneum after surgery. Interventions: The patient underwent resection of the large retroperitoneal tumor. Outcomes: The patient returned 20 months later with tumor recurrence in the retroperitoneum. She was enrolled in a clinical trial for sulfatinib, and the mass was considerably reduced in size after 4 months. During a nearly 1.5-year follow-up, the mass gradually became slightly enlarged. The expression of somatostatin receptor 2 (SSTR2) was detected, and somatuline was administered as the current treatment. Lessons subsections: When a retroperitoneal mass presents as a well-defined cystic or solid hypervascular mass with a fibrous capsule, a primary retroperitoneal NET should be considered in the differential diagnosis.

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“…In some cases, they can stop and cause obstruction and visceral perforation, mostly when they reach an area where anatomical narrowing is present, such as the cricopharyngeus muscle, aortic arc, left main stem bronchus, gastroesophageal junction, pylorus, duodenal sweep, ileocecal valve and anus [1]. Obviously, a sharp object, like a fishbone, carries a higher risk of perforation, up to 35% [7]: Typical clinical symptom is acute abdominal pain and differential diagnosis includes appendicitis, diverticulitis and peptic ulcer perforation.…”

Section: Discussionmentioning

confidence: 99%

“…Foreign body ingestion is a very common reason for access to the Emergency Department (ED): Most of foreign bodies don't need any procedure and leave gastrointestinal tract spontaneously; about 20% require endoscopic removal; surgery is needed in about 1% of cases [1].…”

Section: Introductionmentioning

confidence: 99%