Endometrial endometrioid adenocarcinoma with lymphangiomyomatosis of pelvis lymph node: clinicopathological analysis / 临床与实验病理学杂志

Purpose To study the clinicopathological features of lymphangiomyomatosis (LAM) of pelvis lymph node.Methods A patient with endometrial endometrioid adenocarcinoma and LAM was analyzed including clinical data and pathological features.HE and immunohistoehemistry of EnVision stainings were used,and the literatures were reviewed.Results Well-moderately differentiated endometrioid adenocarcinoma could be observed in the endometrium.Proloferation of LAM cells were seen in the capsule and medulla of the pelvic lymph node.The LAM cell was spindle,epitheliod and polygonal cells with oxyphilic or clear cytoplasm which arranged surrounding lacunes.The LAM cells showed no atypia and mitosis could not seen.The tumor cells showed diffusely positive for SMA,Caldesmon,desmin,vimentin,ER and PR,the cells lining the lacunes were positive for CD34 and D2-40.The epitheliod cells were positive for HMB-45 and negative for Melan-A.The Ki-67 immunostaining showed a proliferation index of ＜ 1％.Conclusion LAM is an uncommon neoplastic multisystem disease that affects the lungs mostly.Endometrial endometrioid adenocarcinoma with LAM of pelvic lymph node is extremely rare.The diagnosis can be made according to the histological characteristics and immunohistochemical features.Moreover this conclusion will provide the clinicopathological materials for the future study about LAM.

High resolution CT findings of pulmonary lymphangioleiomyomatosis / 中国医师进修杂志

Objective To discuss the high resolution CT (HRCT) characteristics of pulmonary lymphangioleiomyomatosis (PLAM),and improve the diagnosis ability of the disease.Methods The pulmonary HRCT changes in 13 patients with pathology proved PLAM were retrospectively analyzed.Results The reported 13 cases of PLAM were all female,the average onset age was 40 (28-57) years old.HRCT showed bilateral,diffuse thin walled cysts throughout both lungs in 11 cases.The cystic air spaces ranged in diameter from a few millimeter to 2 cm,the thickness of the cyst walls was 1-2 mm,air bag cavity surrounded by normal lung tissue.Multiple well-defined cystic air spaces surrounded by uniformly thin wall in 2 cases,no normal lung tissue between the air cavity.One case had complications of left side pleural effusion and lymph nodes of multiple retroperitoneal.Conclusion The HRCT findings of PLAM has diagnostic performance,and it should be used as the first choice for the diagnosis of the disease.

Lymphangiomyomatosis discovered by massive hemoptysis during general anesthesia: A case report / 대한마취과학회지

Lymphangiomyomatosis (LAM) is a rare lung disease that is characterized by the progressive proliferation of atypical smooth muscle-like cells, which leads to severe respiratory impairment and death. Dyspnea, cough, recurrent pneumothorax, and hemoptysis are the most common clinical symptoms of LAM. We report a 29-year-old female patient with massive hemoptysis during laparoscopic gynecologic surgery under general anesthesia, who was diagnosed with pulmonary LAM.

Imaging diagnosis of the lymphangioleiomyomatosis / 中华放射学杂志

Objective Toinvestigatethevalueofimagingdiagnosisofthe lymphangioleiomyomatosis( LAM ).MethodsFifteen patients with LAM confirmed by pathological assessment were analyzed retrospectively for radiologic findings.They had chest radiograph, chest highresolution CT (HRCT),abdominal CT, direct lymphangiography(DLG), chest CT and abdominal CT after DLG.Results Chest radiograph findings included normal (1),increasing of lung markings (3),disseminated honeycomb or reticular pattern ( 11 ), pneumothorax ( 2 ), and pleural effusion ( 14 ). Chest conventional CT and HRCT showed typical imaging manifestation of PLAM in all cases, including sporadic or disseminated cysts in bilateral lungs. According to the grading standard of pulmonary disease made by Avila et at, there were 3 cases in grade Ⅰ , 5 cases in grade Ⅱ and 7 cases in grade Ⅲ . Fourteen of 15 patients with LAM had positive abdominal CT findings in retroperitoneum and pelvic cavity. Common abdominal CT findings included cystic lymphangioma in 9 of 14 patients, lymphangiomyoma in 13 and both coexisting in 7.One of the14patients alsohadhepaticlipomaandangiomyolipomas.Onepatienthadrenal angiomyolipomas; and one patient had hysteromyoma. All 15 cases underwent DLG, 1 cases had lymphatic obstruction in the lumbar 3 level, the remaining 14 cases had varying degrees of thoracic duct stenosis, or obstruction. Neck trunk, subclavian trunk and bronchial trunk showed lymphatic reflux. On post-DLG CT,thoracic duet outlet obstruction was not demonstrated in 3 cases, the remaining 12 cases showed thoracic outlet obstruction, consistent with the DLG findings.Conclusion HRCT is a useful diagnostic method showing characteristic findings of PLAM. MSCT can help to detect abdominal LAM. DLG and MSCT after DLG have value in displaying obstruction site of thoracic duct or lymphatic trunks and provide guidance for operative treatment.

Perivascular Epithelioid Cell Tumor (PEComa) of Abdominal Cavity from Falciform Ligament: A Case Report

We present a case of perivascular epithelioid cell tumors (PEComas) in the abdominal cavity at the falciform ligament. A 30-yr-old Korean man visited to hospital for the evaluation of a growing, palpable abdominal mass. He had felt the mass growing over 6 months. There was no family or personal history of tuberous sclerosis. The resected specimen showed a mass of 8.0x7.0x5.5 cm in size. Histological examination showed sheets of spindle-to-epithelioid cells with clear-to-eosinophilic cytoplasm. Immunohistochemically, tumor cells were positive for HMB-4 (gp100) and smooth muscle actin. They were also positive for the S-100, which is a marker of neurogenic and melanocytic tumors. Patient was treated with radical resection of tumor without any adjuvant therapy. He is well and on follow-up visits without tumor recurrence.

Lymphangiomyomatosis Arising in the Pelvic Cavity: A Case Report

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 9.7x4.2 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.

Retroperitoneal Lymphangiomyoma in a Patient with Pulmonary Lymphangiomyomatosis: Case Report

Retroperitoneal lymphangiomyoma is rare abdominal finding of lymphangiomyomatosis (LAM). We report here on a case of retroperitoneal lymphangiomyoma and this is the first case that? been seen in the Korean literature. Retroperitoneal lymphangiomyoma associated with pulmonary LAM has rarely been reported in radiologic literature. The charateristic findings of this case are a prominent solid component and retroperitoneal lymphadenopathy mimicking a malignant tumor. A 45-year-old woman was admitted for further evaluation of a lower abdominal mass that could be palpated for 2 weeks. US findings showed relatively well-defined mass with septated cystic portions and echogenic solid portions in the lower abdomen. CT findings revealed a well-demarked retroperitoneal mass with septated cystic portions and enhancing solid portions at the right lower abdomen, and there were multiple retroperitoneal lymphadenopathy at the lower abdomen. The patient underwent a mass excision. The pathologic findings were retroperitoneal lymphangiomyoma with multiple lymph node involvement.

Imaging Diagnosis of the Lymphangiomyomatosis (A Report of 2 Cases) / 实用放射学杂志

Objective To improve the imaging recognition of the lymphangiomyomatosis.Methods The manifestations of 2 patients with lymphangiomyomatosis confirmed by pathological assessment were analyzed retrospectively and relevant literature were reviewed.Results Multiple,well-defined and thin-walled cysts were found on the chest HRCT.The size of the cystic airspace was different,and the wall of cysts ranged being fainted perceptible to near l mm in thickness.The retroperitoneal lymphangiomyomatosis were found in the 2 patients on CT images,and some lymphadens were confluence.The diameter of the largest lymphadens was 25 mm,no enhancement were found on contrast-enhanced CT scan.The right renal angioleiomyoma and retroperitoneal leiomyoma were presented in one patient,which were moderately homogeneous enhanced on contrast-enhanced CT scan.Conclusion Lymphangiomyomatosis is a rare lymphangial disease with smooth muscle abnormal hyperplasia.The diffuse cysts in bilateral lungs were its characteristic changes on CT images.Lymphangiomyomatosis can incorporate with renal angioleiomyoma,retroperitoneal leiomyoma and lymphangiomyomatosis.

An Unusual Manifestation of Pulmonary Lymphangiomyomatosis: Airspace Consolidation Masking Cystic Lung Lesions

We report a case of pulmonary LAM occurring in a 24-year-old pregnant woman in whom large areas of air-space consolidation partly masked interstitial lung disease on plain radiography. For correct dignosis, high resolutionCT was considered to still be a potent method for the disclosure of typical interstitial lung changes, including thin-walled cysts. Follow-up CT showed progressive worsening of these interstitial lung lesions.