Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 32-year-old Japanese housewife with a 2-year history of retroperitoneal and pulmonary lymphangiomyomatosis had a sudden onset of severe abdominal pain. Chest X-ray and plain abdominal films showed free air under the diaphragm and a small pneumothorax in the left lower pleural space. Emergency laparotomy revealed no perforated viscera, but there was a rupture in the mid-portion of the left diaphragm. It appears that increasing pressure in the left pneumothorax burst the left diaphragm leading to pneumoperitoneum.
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PMID:Pneumoperitoneum in a patient with lymphangiomyomatosis. 174 58

Lymphangioleiomyomatosis is a rare disease with proliferation of smooth muscle cells within the lymphatics, mediastinal and retroperitoneal lymph nodes and in the lungs. The clinical symptoms are increasing dyspnea, chylous effusion, intestinal obstruction and thoracic or abdominal pain. The authors report the case of a 42-year-old woman who primarily suffered from thoracic pain, dyspnea and chylous effusion. In further examinations we discovered a leftsided retroperitoneal tumor and a tumor in the mesentery. The diagnostic difficulties experienced are described and the necessity of explorative laparotomy for definite diagnosis is demonstrated. Furthermore, the article provides a review of the latest developments in pathology, diagnostics and therapy.
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PMID:[Lymphangioleiomyomatosis--a rare disease of the lymphatic system]. 876

Symptomatic uterine lymphangioleiomyomatosis (LAM) simulating high-stage uterine sarcoma in a patient with tuberous sclerosis complex is reported. A 49-year-old female presented with abdominal pain and anemia. Preoperative workup revealed a uterine mass and a large amount of peritoneal free fluid and possible metastatic implant along the lateral edge of the liver. The patient also had a large right pleural effusion. A fungating anterior uterine fundal mass with apparent perforation and intraabdominal hemorrhage was found on laparotomy. A portion of the mass was excised and initially interpreted as an endometrial stromal sarcoma. Microscopic examination revealed multiple vascular epithelioid smooth muscle proliferations in the uterus and serosal surface of the fallopian tube and periaortic lymph node lymphangioleiomyomas. The uterine, fallopian tube, and nodal lesions were positive for smooth muscle actin, desmin, and HMB-45, findings characteristic of LAM. Additional examination of the patient revealed stigmata of tuberous sclerosis complex. Although uterine LAM is uncommon, it may be associated with pelvic and/or abdominal symptoms and may simulate a primary uterine mesenchymal neoplasm.
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PMID:Lymphangioleiomyomatosis of the uterus simulating high-stage endometrial stromal sarcoma. 894 80

We describe the lesions of extrapulmonary lymphangioleiomyomatosis (LAM) affecting the lymph nodes of the mediastinum and retroperitoneum in 22 women (mean age +/- SD, 42.4+/-10.5 years). In most of these patients, the diagnosis of extrapulmonary LAM preceded that of pulmonary LAM, usually by 1 to 2 years. Eleven patients had distinct symptoms, including chylous pleural effusion and/or ascites, abdominal pain, and palpable abdominal masses. In the other 11 patients, the masses caused no symptoms. Well-circumscribed, encapsulated masses, measuring up to 20 cm in size, occurred in the mediastinum in 2 patients, the upper retroperitoneum in 15, extensive areas of the retroperitoneum in 2, and the pelvis in 3. The masses exceeding 3 cm in diameter contained large, multiple cysts filled with yellow-tan chylous fluid. Histologically, the masses were characterized by a proliferation of smooth muscle cells (LAM cells) arranged in fascicular, trabecular, and papillary patterns, which were associated with slit-like vascular channels. The LAM cells varied from small, spindle-shaped cells to large epithelioid cells. Immunohistochemical studies showed a strong reactivity of most LAM cells for alpha-smooth muscle actin and smooth muscle myosin heavy chain and a weak to moderate reactivity of a lesser number of cells for desmin and nonmuscle myosin heavy chain II-B. A reaction for HMB-45 and estrogen and progesterone receptors was observed mainly in epithelioid LAM cells. These patterns of reactivity are similar to those observed in pulmonary LAM. However, the chylous cysts are not a feature of pulmonary LAM and are thought to result from obstruction of lymphatics.
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PMID:Extrapulmonary lymphangioleiomyomatosis (LAM): clinicopathologic features in 22 cases. 1107 Jan 17

Lymphangioleiomyomatosis (LAM) is a rare idiopathic disease characterized by hamartomatous smooth muscle proliferation of the lymph node, lymphatics, blood vessels as well as airways within the lungs, mediastinum and abdomen. It exclusively affects women during the reproductive period. Though extrapulmonary manifestations have been reported, the initial presentation of LAM as abdominal pain is extremely rare. To our knowledge, there are only three cases with LAM presenting first with abdominal symptoms so far [Chest 106 (1994) 267; Eur J Radiol 14 (1992) 192; Eur J Surg 157 (1991) 36]. We describe a case of LAM suffering from abdominal pain followed by pulmonary symptoms and the diagnosis was not made until pathohistological examination.
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PMID:Lymphangioleiomyomatosis initially presenting with abdominal pain: a case report. 1272 53

In this study, we investigated HMB-45 expression in epithelioid uterine leiomyosarcomas with clear cell areas. From 12 epithelioid leiomyosarcomas, we selected 5 that had: 1) clear cell areas and 2) spindle cell areas that were at least focally positive for desmin and caldesmon. The patients' ages ranged from 47 to 82 years (mean 64 years). Presenting symptoms were uterine bleeding (three), abdominal pain (one), and a pelvic mass (one). There was no history of tuberous sclerosis or lymphangioleiomyomatosis. One patient had stage II disease, one stage III, and three stage IV. All were treated with total abdominal hysterectomy and bilateral salpingo-oophorectomy. Two received radiotherapy, and three were also treated with chemotherapy. The tumors ranged in size from 4 x 3 x 3 cm to 10 x 7 x 6 cm; all had significant cellular atypia, areas of coagulative necrosis, and between 10 and 90 mitoses per 10 high power fields. Vascular invasion was seen in three cases. The epithelioid component varied from 50% to 90% in each case; and the percentage of clear cells was < 1% in one case, 5% in one case, and 10% to 80% in three cases. Smooth muscle actin and desmin were positive in all cases. Four cases were positive for HMB-45 only in the clear cell areas. The tumor with < 1% of clear cells was negative for HMB-45. All were negative for S-100 and c-kit. Three patients died of disease at 9, 30, and 32 months; one patient is alive with progressive disease at 6 months, and one patient (stage II disease) is alive with no evidence of disease at 8 months. Unequivocal uterine epithelioid leiomyosarcomas may have clear cells positive for HMB-45. These tumors might belong to the group of lesion designated as PEComas; however, it is advisable to designate them as uterine leiomyosarcomas. In uterine smooth muscle tumors, some epithelioid cells most likely undergo clear cell changes and become positive for HMB-45. It would be advisable to perform this stain in all epithelioid smooth muscle tumors of the uterus.
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PMID:Uterine epithelioid leiomyosarcomas with clear cells: reactivity with HMB-45 and the concept of PEComa. 1504 15

Lymphangioleiomyomatosis (LAM) is a rare disease usually occurring in young women of child-bearing age. It is characterized by a distinctive proliferation of lymphatic smooth muscle cells, especially occurring in the pulmonary parenchyme. The majority of primary LAM occurs in the lung, but there are a few reports of extrapulmonary cases. We report a case of a 21-yr-old female who first complained of low abdominal pain and was referred from a local clinic with the impression of an ovarian cyst. Gynecologic ultrasonography revealed a large posterior pelvic mass with an irregular echogenicity measuring 9.7 x 4.2 cm in size. Pelviscopy showed a large, thin walled, partly cystic, pelvic mass. The mass was partly removed. Microscopically, the mass was characterized by a haphazard proliferation of smooth muscle cells arranged in fascicular, trabecular, and papillary patterns around a ramifying network of endothelium-lined spaces. The cells were plump or epithelioid with abundant eosinophilic cytoplasm and showed a positive reaction for both alpha-smooth muscle actin and HMB-45 antigen. Surgical and pathological findings were consistent with pelvic retroperitoneal LAM. Despite the numerous treatment attempts, the patient suffered from intractable chylous ascites and developed pulmonary LAM and died due to severe respiratory distress.
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PMID:Lymphangiomyomatosis arising in the pelvic cavity: a case report. 1622 74

A 40 year-old 2nd gesta pregnant woman (34.5 weeks of amenorhea) was admitted to hospital for abdominal pain and arterial hypotension which were rapidly related to a retroperitoneal haematoma due to left kidney bleeding. Emergency cesarean delivery under general anaesthesia was undertaken because of foetal distress. Exploration of the retroperitonal space after foetal extraction confirmed the presence of a large haematoma and abnormal left renal morphology. The retroperitoneal space was drained without any further intervention. Subsequently, abdominal and thoracic computerised tomographic examination showed bilateral dysplasia of the kidneys and pulmonary cysts consistent with the diagnosis of renal angiomyolipoma and pulmonary lymphangioleiomyomatosis. The case report is of interest because of the circumstances of discovery of the disease and because nephrectomy was not necessary to control the bleeding of the left kidney. Six months after the incident the patient and the child are in good condition.
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PMID:[Renal angiomyolipoma rupture during pregnancy]. 1699 72

Lymphangioleiomyomatosis (LAM) is a rare progressive disease of unknown etiology that typically affects women of childbearing age. It is characterized by an abnormal proliferation of smooth muscle cells causing gradual obstruction of small airways, frequently resulting in respiratory failure and death. While LAM is predominantly a lung disorder, we report a case of retroperitoneal LAM in a patient who had no evidence of pulmonary involvement. A 59-year-old female presented with postmenopausal bleeding and no other complaints. She was found to have a low attenuation retroperitoneal mass on abdominal and pelvic computed tomography (CT) scan suspicious for lymphoma. CT-guided biopsy was nondiagnostic. Laparoscopic resection of some of the enlarged retroperitoneal lymph nodes confirmed the diagnosis of LAM. This case is an atypical presentation of LAM. The disease typically presents in premenopausal women, with the initial site of involvement being the lungs and mediastinum. In rare cases, such as this of extrapulmonary LAM, patients typically present with a palpable abdominal mass, abdominal pain, or chylous ascites. As in our case, radiographic findings can mimic malignancies such as lymphoma. Laparoscopic lymph node biopsy is a valuable tool in these situations of diagnostic dilemma.
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PMID:Primary retroperitoneal lymphangioleiomyomatosis in a postmenopausal woman: a case report and review of the literature. 1736 25

A previously healthy 28-year-old woman presented to the emergency clinic with acute severe abdominal pain and high fever. A diagnostic laparoscopy was performed, during which a large retroperitoneal tumour was found. A CT-scan of the abdomen and thorax confirmed the presence of a retroperitoneal mass but also revealed multiple renal angiomyolipomas and extensive cystic lesions in all lung fields. Based on these findings, the diagnosis lymphangioleiomyomatosis (LAM) was suspected, and later confirmed by histological examination of a biopsy specimen. The acute abdomen and fever appeared to have been caused by a Streptococcus agalactiae infection of the retroperitoneal lymphangiomyoma, which was treated with intravenous antibiotics. LAM is a very rare disease affecting mostly women of childbearing age and presenting almost exclusively with pulmonary symptoms. This is the first description of LAM presenting with an acute surgical abdomen and fever due to infection of a lymphangiomyoma.
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PMID:[Acute abdominal pain and fever in a young woman as the first symptoms of lymphangioleiomyomatosis]. 1824 Jul 60


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