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At least 19 recordsLinked to original sources
PubMedDate not supplied

Intraabdominal lymphangiomyoma in an infant with protein-losing enteropathy and hemihypertrophy.

Lymphangiomyoma is an extremely rare tumor occurring exclusively in women of reproductive age. The tumor is characterized by proliferation of immature smooth muscle along the lymphatic vessels of the abdomen, thorax and lung. Although lymphangiomyoma has been reported in a young girl and a girl infant, none has been reported in boys. We report herein a case of lymphangiomyoma in a two-year-old boy. The unusual presentation in this patient was that the tumor arose from the small bowel mesentery without any evidence of lung involvement. The tumor was extirpated and lymphangiomyomatosis was confirmed pathologically.

Anastomosis, Surgical↗
PubMed1978

Lymphangiomyoma of the thoracic duct with previously undescribed epithelial tubules.

A case of mediastinal lymphangiomyoma with tubules covered with epithelium is reported. The epithelium of these tubules is morphologically identical to that of the bronchi. It is the first time that epithelial, presumably pulmonary, elements have been described in an extrapulmonary lymphangiomyoma. It is concluded that the present case of mediastinal lymphangiomyoma suggests a hamartomatous malformation. Whether this applies in general to lymphangiomyomatosis still remains a matter of discussion.

Adult↗
PubMed1998

Mediastinal lymphangiomyoma in a child: report of a case.

Lymphangiomas and lymphangiomyomas are benign abnormal collections of lymphatic vessels which form a cystic mass. The mediastinum is an uncommon location for these hamartomas in children, but may be life-threatening. We report herein the case of a 23-month-old boy who presented with stridor and a history of recurrent respiratory tract infections. Soon after his admission he suddenly developed respiratory failure and was found to have a multiloculated, cystic mediastinal mass which was initially thought to be a lymphangioma. The mass was extensively infiltrative, and histological examination showed prominent smooth muscle proliferation in the walls of the cysts, confirming a diagnosis of lymphangiomyoma. The terminology and relevant literature on the topic are reviewed following the presentation of this case.

Humans↗
PubMed1989

Protein-losing enteropathy in an infant with a lymphangiomyoma.

A 3-month-old girl with intractable diarrhoea had protein-losing enteropathy secondary to a lymphangiomyoma. This is the first reported case of lymphangiomyoma in an infant. As enteral feeds were not tolerated, she was maintained on parenteral nutrition for 2 months. Pancreatic enzyme supplementation produced a rapid clinical improvement and normalization of serum albumin level. Weaning was subsequently tolerated and the vitamin, mineral, and trace element deficiencies improved. Subsequent recurrence was associated with normal pancreatic function and has proved refractory to treatment.

Adult↗
PubMed1993

[Lymphangiomyoma of the thoracic duct].

From the right superior mediastinum of a 31-year old woman the authors extirpated a lymphangiomyoma originating from the thoracic duct. The clinical feature and pathology of this rare lesions are discussed. This case is the first successfully removed thoracic duct lymphangiomyoma in Hungary.

Adult↗
PubMed1977

Lymphangiomyoma.

A case report is presented of the incidental finding of a lymphangiomyoma in a 50-year-old woman who underwent total hysterectomy for squamous cell carcinoma of the cervix. The condition seems to occur only in women after puberty and so far, has been mainly reported in association with chylothorax or chylous ascites. There is no evidence of any clinical complication more than 2 years after removal of the lesion. The lesion is most likely a hamartoma although the term lymphangiomyoma is acceptable. The term lymphangiomyomatosis is best reserved for the fully developed clinico-pathological syndrome.

Carcinoma, Squamous Cell↗
PubMed1992

Giant thoracoabdominal lymphangioma with features of lymphangiomyoma.

A 15-year-old girl who presented with cough and dyspnea was found to have a mediastinal tumor that clinically resembled a lymphangioma. The tumor was unusual for its large size and its histologic features, which showed smooth muscle proliferation, generally considered a feature of lymphangiomyoma.

Adolescent↗
PubMed1989

[Dietary treatment of patients with lymphangiomyoma].

A case of lymphangiomyoma, manifested by chylous ascites, in a 32 year-old woman is presented. Treatment with dietary fat restriction supplemented with medium chain triglycerides was successful, and the patient has remained free from chylous effusions for nearly two years.

Adult↗
PubMed1995

[Aneurysmal iliac lymphangiomyoma. Report of a case].

Single lymphangiomyoma, rarely encountered, is a form of lymphangiomyomatosis with characteristic proliferation of smooth muscle cells in lymph nodes and vessels. We observed a case in a 47-year-old woman with no past medical history. An echography performed for menorrhage revealed a single iliac localization in the right pelvis. The precise pre-operative diagnosis was difficult, but the pathology report formally identified the tumor as an isolated form without thoracic involvement. Unlike lymphangiomatosis itself which involves pulmonary lesions and which may require complementary treatment, total exeresis of a single tumour is the only treatment necessary, as was confirmed in this case.

Bone Neoplasms↗
PubMed1975

Cystic lymphangiomyoma of the colon causing protein-losing enteropathy.

This report documents a case of cystic lymphangiomyoma of the sigmoid colon in a 35-year-old woman who presented with symptoms of a protein-losing enteropathy. This case was unique in that it involved not only the colonic wall and mesentery but also extended into the retroperitoneum. Surgical excision of the affected segment has resulted in reversal of hypoproteinaemia and return to normal of the excessive faecal loss of 51Cr-labeled protein over a 2-year follow-up period.

Adult↗
PubMed2000

[Localized lymph node lymphangiomyoma. Description of 2 cases].

Lymphangioleiomyomatosis is a rare disease characterized by an immature-appearing smooth muscle proliferation in the lung and along axial lymphatics. Rarely it is limited to lymph nodes, without extranodal disease. Two cases of localized nodal lymphangiomyoma in a 48 and 54 year-old women are presented: in both cases the lesion was limited to pelvic lymph nodes and it was an incidental finding during staging for tumours of the gynaecological tract.

Carcinoma, Endometrioid↗
PubMed1997

Retroperitoneal lymphangiomyoma in an infant.

Lymphangiomyomatosis (LAM) is a benign tumor-like lesion of lymphatic vessels with unknown etiology. 80 cases of LAM are presently described. So far, it appears that only women in their reproductive years seem to be affected. Here, we describe the first LAM in an 11-months-old infant with a presumably local form of LAM. This leads us to consider the possibility that this tumor-like lesion may originate from a hamartomateous malformation. The diagnosis is based on pre-operative sonography and CT, as well as on histological and immunohistochemical examination of the tumor.

Female↗