A diagnosis of lymphangioleiomyomatosis in a pregnant woman presenting with a retroperitoneal mass

Abstract

A 30 year old white primigravida presented with abdominal pain, fatigue, general malaise, loss of weight and a rise in temperature at 10 weeks of gestation. Her medical history was unremarkable. Ultrasonography of the abdomen showed a mass with central translucency, with the appearance of an abscess or a tumour with central necrosis. Magnetic resonance imaging confirmed the mass on the right side of the bifurcation of the large vessels. On the suspicion of a retroperitoneal lymphoma, a biopsy was performed under ultrasonic guidance. Histology revealed cells with no mitotic figures that, on additional histochemical examination showed individual entwinement by reticuline fibres. Immunohistochemical examination showed vimentine and actine positive cells, a morphological aspect, strongly suspicious of a leiomyosarcoma. Clinically there were some doubts, because of the difficulty of diagnosing leiom yosarcoma during pregnancy.To obtain more detailed histology, an explorative laparotomy was performed at 15 weeks of gestation. A multifocal retroperitoneal mass (7 x 6 x 5 cm) was found on the right side of the vena cava, with spread to the surrounding tissues. As the mass was not resectable, part of the tumour was removed (3 x 3 x 3 cm). Histology revealed small nodules of smooth muscle proliferation, characteristic of lymphangioleiomyomatosis. There was delay in post-operative recovery because of severe dyspnoea.Pre-operatively, the X-ray of the thorax suggested some old lung fibrosis, but further post-operative review of this X-ray revealed fibrosis consistent with lymphangioleiom y omatosi s . Computerised tomography (CT) scanning of the chest showed cystic structures distributed throughout both lungs. Pulmonary function tests showed a massively reduced diffusion capacity and slight airflow obstruction. There was also moderate arterial hypoxaemia (PacOz. 4-5 kPa, Paoz 9-1 kPa, S h 2 95%). The patient declined the possibility of terminating the pregnancy at 16 weeks of gestation. During the rest of her pregnancy, she required two litres of oxygen per minute by nasal cannula. At 39 weeks of gestation, induction of labour was started because of increasing dyspnoea and eventually a caesarean section was performed because of a lack of progress in the first stage of labour. A healthy female infant was born, weighing 3160 g. There was a decrease in dyspnoea postpartum, with more appropriate recovery than after the laparotomy during pregnancy. The woman breastfed her daughter. Owing to poor pulmonary function test results two months postpartum, she was prescribed oxygen continuously and started to use 200 mg medroxyprogesterone daily. The abdominal mass remained unchanged on ultrasound examination in the postpartum period. One and a half years later a CT of the abdomen showed no progression of the retroperitoneal mass; local treatment of this mass was not offered, because there was no obstruction or pain. Her pulmonary function is still deterioriating slowly. Lung transplantation will be considered. DiscussionLymphangioleiom...

Pulmonary Lymphangioleiomyomatosis (LAM): Examining Oral Contraceptive Pills and the Onset of Disease

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Recurrent pneumothorax in a young female with pulmonary lymphangiomyomatosis: A case report and overview of literature

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Retroperitoneal lymphangiomyomas presenting as a pelvic mass

“…There are only four case reports in the literature of patients with LAM presenting with abdominal symptoms (6–9). Two had pulmonary LAM with concomitant pelvic or abdominal lymphangiomyomas at presentation (6,7), and one was diagnosed at her gestational age of 15 weeks with subsequent successful delivery at 39 weeks (7). The other two had abdominal symptoms at presentation, diagnosed as pulmonary LAM after 3 weeks to 1 year of an initial diagnosis of abdominal or pelvic lymphangiomyomas (8,9).…”