Lymphangioleiomyomatosis: solitary abdominal manifestation (2009: 9b)

Possekel, Anne-Kristin; Katenkamp, D.; Brambs, Hans-Juergen; Pauls, Sandra

doi:10.1007/s00330-008-1275-4

Cited by 6 publications

(3 citation statements)

References 18 publications

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“…Lymphangioleiomyoma is most commonly localized in the retroperitoneal space and the uterus and in surrounding lymph nodes [6,24,27]. Moreover, E-LAM foci developing in the mediastinum [36], supraclavicular lymph nodes [37,38], liver [39], walls of the small and large intestine [40,41], pancreas [26,42], mesentery [43] have been described. E-LAM masses can reach very large sizes -cases of tumors with the greatest length 15-22 cm have been described [18,[44][45][46] -and can extend along lymph vessels -coming from the retroperitoneal space to the chest [19], and further even along the neck along with the sternocleidomastoid muscle [47].…”

Section: Anatomy and Clinical Picturementioning

confidence: 99%

Rozpoznanie i leczenie limfangioleiomiomatozy (LAM) z grupy PEComa

et al. 2021

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Lymphangioleiomyomatosis (LAM) is a rare, proliferative lung disease, leading to progressive damage of their structure and is a member of the PEComa neoplasm family (perivascular epithelioid cell tumors). In the patients, solid-cystic masses described as lymphangioleiomyoma or extrapulmonary LAM (E-LAM) can occur. E-LAM foci have been described in the mediastinum, supraclavicular lymph nodes, the liver, walls of the small and large intestine, the pancreas, mesentery. E-LAM masses can attain very large sizes -tumors 15-22 cm long have been described. On the basis of positive results of clinical trials sirolimus, a drug from the group of mTOR kinase inhibitors, was registered by the Food and Drug Administration (FDA) in May 2015 as the first and currently only drug for systemic LAM therapy. Sirolimus use is recommended in patients with LAM, accompanied by rapidly progressing deterioration of respiratory system function or FEV 1 ≤ 70% predicted value and in patients with pleural lymph exudate before applying invasive methods of treatment.

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Section: Anatomy and Clinical Picturementioning

confidence: 99%

Rozpoznanie i leczenie limfangioleiomiomatozy (LAM) z grupy PEComa

et al. 2021

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“…However, to the best of our knowledge, the occurrence of LAM in the liver has yet to be reported. Only one case of abdominal LAM that presented multiple cysts involving the liver was described by Possekel et al (11) in a 23-year-old patient. The present case is the first extrapulmonary LAM presenting as a liver mass without involving other organs.…”

Section: A B C D E Fmentioning

confidence: 99%

Solitary extrapulmonary lymphangioleiomyomatosis of the liver: A case report and literature review

Fu¹,

Li²,

Li³

et al. 2016

Experimental and Therapeutic Medicine

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Abstract. Lymphangioleiomyomatosis (LAM) is a progressive disorder of unknown etiology that predominantly affects the lungs, and the resulting respiratory failure can be fatal. Extrapulmonary LAM is a rare disease that often occurs concurrently with pulmonary LAM. Usually, extrapulmonary LAM presents as a localized, well-circumscribed mass. Due to the rare occurrence of extrapulmonary LAM and its atypical location, extrapulmonary LAM is often difficult to diagnose prior to surgery. The present study describes the clinicopathological features of primary liver LAM in a 26-year-old woman. To the best of our knowledge, the present study presents the first case of a primary solitary extrapulmonary form of LAM in the liver. IntroductionLymphangioleiomyomatosis (LAM) is a progressive disorder that predominantly affects the lungs, and the resulting respiratory failure can be fatal (1). The typical histology of LAM shows a proliferation of smooth muscle arranged in fascicular, trabecular, and papillary patterns associated with slit-like vascular channels (2). Pulmonary LAM can occur sporadically or in association with the inherited hamartoma syndrome tuberous sclerosis (TSC) (3,4).Extrapulmonary LAM is a rare disease that is usually presented as a localized, well-circumscribed mass (5). The primary sites for LAM include the pancreas, retroperitoneum, pelvis, mediastinum, kidney hilus, uterus and mesentery (6). The clinical features of extrapulmonary lymphangioleiomyomatosis are a palpable abdominal mass, abdominal pain, and chylous ascites (7). Differential diagnoses should include lymphoma, schwannoma, paraganglioma at the para-aortic area, and metastatic tumors in the lymph nodes (2). Due to the rare occurrence of extrapulmonary LAM and its atypical location, extrapulmonary LAM is often difficult to diagnose, especially prior to surgery. To the best of our knowledge, the present study reports the first case of primary LAM presenting as a liver mass. Case reportCase summary. The present study was approved by the Ethics Committee of the Qingdao University Medical College (Qingdao, China). Written informed consent was obtained from the patient's family. In 2009, a 26-year-old woman presented a liver lesion of ~6 cm diameter during a routine examination at Jiaozhou People's Hospital of Qingdao (Qingdao, China). The liver lesion was diagnosed as a liver cyst following a computed tomography (CT) scan of the abdomen. The patient exhibited no clinical symptoms until April 2012. Enhanced abdominal CT scans showed that the mass was located in the left lateral hepatic lobe with 5.7x7 cm enhancement and uneven density (Fig. 1). The radiological differential diagnosis included left lateral hepatic adenoma or focal nodular hyperplasia. Subsequently, a hepatic lobectomy was performed in May, and the patient was discharged after one week.Pathologic findings. Gross examination demonstrated that the liver resection specimen measured 15x10x4 cm. After the specimen was cut longitudinally into 1-cm lobe sections, the liver lobe section exh...

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“…However, extrapulmonary LAM rarely occurs with or without subsequent involvement of the lung, and several cases of extrapulmonary LAM involving abdominal or pelvic retroperitoneal organs have been reported in the radiology literature. [2][3][4][5][6][7] We describe a case of histologically proven retroperitoneal LAM in a female patient with sonography, computed tomography (CT), gadolinium-enhanced magnetic resonance imaging (MRI), and positron emission tomography (PET).…”

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confidence: 99%