Extrapulmonary lymphangioleiomyomatosis in pelvic and paraaortic lymph nodes associated with uterine cancer: a report of 3 cases.

We report 3 cases of extrapulmonary lymphangioleiomyomatosis incidentally found in pelvic and paraaortic lymph nodes in association with uterine cancers. Three women, 47-year-old, 59-year-old, and 71-year-old, respectively, had uterine cancers and underwent hysterectomy, bilateral salpingo-oophorectomy, and pelvic and paraaortic lymph node excision. None of the 3 patients had tuberous sclerosis complex or lymphangioleiomyomatosis in other organs. None had any history of extrinsic hormonal administration. The postoperative pathologic diagnoses were uterine cervical squamous cell carcinoma for the first patient and endometrioid adenocarcinomas for the second and the third patients. Besides these malignant lesions, all 3 patients showed spindle cell proliferation, 2 to 5 mm in size, in 1 to 8 foci of the pelvic and paraaortic lymph nodes. The spindle cells having small polygonal nuclei and inconspicuous nucleoli with palely eosinophilic cytoplasm, reminiscent of immature smooth muscle cells, proliferated in nested and whorling patterns. Neither cellular atypia nor mitotic figures were observed. Immunohistochemically, these spindle cells were positive for α-Smooth Muscle Actin, Desmin, HMB45, Microphthalmia Transcription Factor, Estrogen receptor, and Progesterone receptor. And the network of the vascular-like channels surrounded by these spindle cells was positive for D2-40. From the pathologic and immunohistochemical findings, the spindle cell proliferation in the lymph nodes is best interpreted as lymphangioleiomyomatosis.

Pseudo-Meigs Syndrome Caused by a Giant Uterine Leiomyoma with Cystic Degeneration: A Case Report.

Pseudo-Meigs syndrome is defined as secondary accumulation of ascites and hydrothorax associated with a pelvic tumor other than benign ovarian tumors such as fibroma, which usually resolve after surgical removal of the tumor. Here we report a case of pseudo-Meigs syndrome caused by a giant uterine leiomyoma, which was initially suspected to be ovarian cancer. A 37-year-old nulliparous woman presented with a 5-month history of abdominal distension and anorexia. Abdominal ultrasonography revealed a giant cystic lesion and solid mass in the peritoneal cavity, along with plentiful ascites. Chest X-ray images showed a small pleural effusion on the right side. The patient was referred to our hospital for treatment of suspected ovarian cancer and peritonitis carcinomatosis. Although serum CA125 level was elevated (up to 331.8 U/mL), magnetic resonance imaging showed a giant sub-serosal uterine leiomyoma with cystic degeneration (27 × 15 × 13 cm). A small dermoid cyst was also detected in the right ovary. Ascites was drained and the patient underwent myomectomy and ovarian cystectomy. The patient had a degenerated leiomyoma with no pathological evidence of malignancy. Because symptoms disappeared postoperatively and serum CA125 returned to normal, without recurrence of ascites, pseudo-Meigs syndrome was diagnosed.

[New development of a cytological examination ordering system at Osaka National Hospital].

Effective and rapid use of cytological data are issue in Japan. We addressed this problem by development of an ordering system for cytological examinations at Osaka National Hospital. This system is located in the department of pathology and is a client-server system that consisted of 1 server and 6 clients. Five of the 6 clients are related to cytology and there are connections to microscopes with digital cameras. One client is for acceptance of cytological specimens at the department of pathology. Through a local area network, 100 Mbps, this system is connected to the hospital information system, which is of the order-entry type. After a clinician orders a cytological examination, these data are sent to both the Accounts and Pathology departments. A small bar-code label is simultaneously printed out, which is stuck on the form. By checking this label at the department of pathology by using a label reader, relevant clinical information is sent to the pathology server. After the cytological diagnosis has been made by senior cytotechnologist and cytopathologist, data on the diagnosis and microscopic images are sent to the hospital information system. Thus, clinicians can review the cytological diagnosis together with the photomicrographs. This new cytology system has brought great benefits to both cytotechnologist and clinicians with regard to the rapid transfer of cytological examination, and it seems to contribute to more advanced and efficient medical care.

Case of Budd-Chiari syndrome 3 months after vaginal delivery.

Budd-Chiari syndrome is a rather unusual clinical situation caused by occlusion of the hepatic vein of inferior vena cava, the classical triad of which are abdominal pain, ascites and hepatomegaly. A 29-year-old gravida 3 para 1 woman delivered an immature male baby weighing 2172 g with an Apgar score of 9 points at 35 weeks and 3 days of gestation. She was transferred to the National Hospital Organization Osaka National Hospital 112 days after delivery due to the sudden development of massive ascites. Magnetic resonance angiography and enhanced computed tomography detected the occlusion by thrombosis of both the middle and left hepatic veins, so she was diagnosed with Budd-Chiari syndrome. Her protein C antigen and activity were 37% and 50%, respectively, corresponding to type 1 protein C deficiency. Conservative treatment by continuous oral treatment of spironolactone (25 mg/day), furosemide (20 mg/day) and prophylactic warfarin (2 mg/day) much improved the ascites.

A case of uterine cervical adenocarcinoma associated with small cell carcinoma.

Extrapulmonary small cell carcinomas are often associated with carcinomas of other cell types. Although a hypothesis that extrapulmonary small cell carcinomas arise from a multipotential stem cell could explain this mixed feature, recent molecular evidence supports another possibility that the small cell component may arise as a late-stage phenomenon in the progression of more organ-typical carcinomas. Here, we report a case of uterine cervical adenocarcinoma containing 30% of small cell carcinoma. Adenocarcinoma was located in the endometrial side of the tumor that was adjacent to the normal cervical region, while small cell carcinoma was located in the periphery of the tumor. The transition from adenocarcinoma to small cell carcinoma was observed in the boundary area. These findings suggest that cervical small cell carcinoma can be differentiated from pre-existing adenocarcinoma and offer further support to the hypothesis that the small cell component arises as a late-stage phenomenon in the progression of more organ-typical carcinomas.

Incidence of postoperative ileus after paraaortic lymph node dissection in patients with malignant gynecologic tumors.

BACKGROUND: Paraaortic lymph node metastasis is an important prognostic factor in gynecologic malignancy. However, paraaortic lymph node dissection (PAND) is not done routinely in Japan because of the difficulty of the procedure and the high incidence of complications. We performed PAND in 217 patients in a 7-year period. In the present study, we focused on the occurrence of postoperative ileus in patients who underwent PAND. METHODS: Two hundred and seventeen patients with malignant gynecologic tumors were operated on at our hospital between January 1995 and August 2001. All patients underwent PAND and pelvic lymph node dissection (114 patients had a radical hysterectomy; 103 patients had a simple hysterectomy). We evaluated postoperative ileus in three categories of severity. RESULTS: The average operation time and blood loss in the patients with radical and simple hysterectomies with PAND were 317 min and 1158 g, and 246 min and 820 g, respectively. The incidence of postoperative ileus was 12.9% (28/217). Although there were no significant differences in the occurrence of ileus between patients with the radical and simple hysterectomies (10.5% vs 15.5%), the incidence of ileus in patients with radical hysterectomy with PAND was significantly higher than that in a control group of patients with radical hysterectomy without PAND (10.5% vs 3.4%). However, in the PAND patients the postoperative ileus was mostly mild or moderate (10 mild cases, 15 moderate cases, and 3 severe cases). Severe ileus occurred in three patients with radical hysterectomy with PAND. Although a repeat operation was necessary for two of these three patients with severe ileus, they recovered uneventfully. CONCLUSION: PAND for malignant gynecologic tumors is a feasible and safe operative procedure, with a low incidence of postoperative ileus.

Adenocarcinomas arising from uterine adenomyosis: a report of four cases.

Adenocarcinomas arising from adenomyosis uteri are rare. This study reports four such cases and characterizes them clinically and microscopically. In all four patients, the endometrial cytology was negative, and MR imaging and ultrasound sonography did not detect the tumors preoperatively. The histological subtypes of the four tumors were endometrioid (one grade 1, one grade 3), serous, and clear cell. In three cases, the adenocarcinomas were present exclusively in the myometrium, and a transition between the carcinomas and the adenomyotic glands was observed in all cases. The eutopic endometrium was normal except in one case in which there was a small focus of invasive carcinoma. In two of four cases, pelvic or paraaortic lymph node metastases were present. In the carcinomas, ER immunoreactivity was not found in any tumor and PR positivity was found in only one tumor. In contrast, p53 immunopositivity was found in three of four carcinomas. Adenocarcinomas arising from adenomyosis are difficult to diagnose preoperatively, and their aggressive behavior in some cases seems to be related to the histological subtype.