Malignant perivascular epithelioid cell tumour of the round ligament mimics leiomyoma on computed tomography.

We report a case of a 45-year-old woman who had a palpable mass in the left lower quadrant of the abdomen. Computed tomography (CT) showed a circumscribed mass in the left round ligament of the uterus. The mass had heterogeneous density and enhancement accompanied by mottled calcification, which was initially identified as a leiomyoma. A histopathological examination revealed a malignant perivascular epithelioid cell tumour (PEComa), which is a rare soft tissue tumour. This case demonstrates that the appearance of malignant PEComa on the CT can mimic leiomyoma, which is the most common tumour of the round ligament. To the best of our knowledge, this is the first report of a CT appearance of this round ligament tumour. The radiological features and differential diagnosis are discussed.

Epstein-Barr virus-associated smooth muscle tumor presenting as a vulvar mass in an acquired immunodeficiency syndrome patient: a case report.

Smooth muscle tumors in immunocompromised patients have a strong association with Epstein-Barr virus (EBV) infection. EBV-associated smooth muscle tumors (EBV-SMT) are considered as a distinct group of smooth muscle tumors with different clinicopathologic features from conventional smooth muscle tumors. A 31-year-old female patient presented with a 2-cm mass at the left labium majus, the clinical diagnosis of which was a Bartholin lesion. She had acquired immunodeficiency syndrome diagnosed 29 months before. Excisional biopsy revealed a cellular tumor composed of round- to spindle-shaped cells with mild to moderate nuclear atypia. The tumor cells were immunoreactive for smooth muscle actin and muscle actin (HHF-35). Evidence of EBV infection was confirmed by in situ hybridization for EBV-encoded small RNA-1. To our knowledge, this is the first case of EBV-SMT presenting as a vulvar mass. EBV-SMT should be included in the differential diagnoses of mesenchymal tumor in patients with immunosuppression and in the differential diagnoses of smooth muscle tumor in uncommon sites, including the vulva.

Posterior approach technique for accessory-suprascapular nerve transfer: a cadaveric study of the anatomical landmarks and number of myelinated axons.

Accessory-suprascapular nerve transfer by the anterior supraclavicular approach technique was suggested to ensure transferrance of the spinal accessory nerve to healthy recipients. However, a double crush lesion of the suprascapular nerve might not be sufficiently demonstrated. In that case, accessory-suprascapular nerve transfer by the posterior approach would probably solve the problem. The aim of this study was to evaluate the anatomical landmarks and histomorphometry of the spinal accessory and suprascapular nerve in the posterior approach. Dissection of fresh cadaveric shoulder in a prone position identified the spinal accessory and suprascapular nerve by the trapezius muscle splitting technique. After that, nerves were taken for histomorphometric evaluation. The spinal accessory nerve was located approximately halfway between the spinous process and conoid tubercle. The average distance from the conoid tubercle to the suprascapular nerve (medial edge of the suprascapular notch) is 3.3 cm. The mean number of myelinated axons of the spinal accessory and suprascapular nerve was 1,603 and 6,004 axons, respectively. The results of this study supported the brachial plexus reconstructive surgeons, who carry out accessory-suprascapular nerve transfer by using the posterior approach technique. This technique is an alternative for patients who have severe crushed injury of the shoulder or suspected double crush lesion of the suprascapular nerve.

Women in a region with high incidence of cervical cancer warrant immediate colposcopy for low-grade squamous intraepithelial lesion on cervical cytology.

The objective of this study was to determine the prevalence of high-grade histologic diagnoses in women who had low-grade squamous intraepithelial lesion (LSIL) on Pap smear in an area with high incidence of cervical cancer. We conducted a retrospective study of 220 women with LSIL cytology undergoing colposcopic examination in Chiang Mai University Hospital between January 1999 and July 2004. The histologic diagnoses, obtained from colposcopically directed biopsy or electrical loop excision after initial colposcopy, showed that 80 (36.4%) women had histologically confirmed high-grade lesions and 11 (5%) women had microinvasive (9) and frankly invasive (2) carcinomas. Overall, 41.5% of women with LSIL on Pap smear had significant underlying lesions, requiring appropriate treatment. In conclusion, in the region with high incidence of cervical cancer, women with LSIL cytology are at increased risk of having underlying high-grade lesions and invasive cancer. Immediate referral for colposcopy is warranted.

The risk of residual neoplasia in women with microinvasive squamous cervical carcinoma and positive cone margins.

The objective was to evaluate the prevalence and factors affecting residual disease in women with cervical microinvasive carcinoma (MIC) with positive cone margins for high-grade lesions and invasive carcinoma. We reviewed histopathology slides of 129 women with MIC who had high-grade lesions or invasive carcinoma at cone margins. These patients underwent hysterectomy following cone biopsy between January 1994 and June 2004. Of the 129 patients, 77 (59.7%) had residual disease in the hysterectomy specimens, in which 57 (44.2%) had residual high-grade lesions. Twenty patients (15.5%) had residual invasive carcinoma: 18 were microinvasive and 2 were invasive. Factors significantly affecting the risk of residual disease included positive postconization endocervical curettage (P= 0.001), positive cone margins for invasive carcinoma (P= 0.003), and depth of stromal invasion >1 mm (P= 0.014). Cox proportional hazards analysis revealed positive cone margins for invasive carcinoma as significant predictor of residual invasive disease (hazard ratio, 3.22; 95% CI 1.21-8.60, P= 0.019) In summary, patients with MIC and positive cone margins for high-grade lesions or invasive carcinoma are at high risk of residual neoplasia. Repeat cone biopsy should be performed to determine exactly the severity of lesion before planning treatment.

Metastatic tumors to the ovaries: a study of 170 cases in northern Thailand.

The cases of malignant ovarian tumors treated at Chiang Mai University hospital between 1992 and 2003 were histologically reviewed. The medical records, the radiologic findings, and the follow-up outcome in the cases suspicious or diagnostic of metastases were reviewed to confirm the diagnosis and to determine the primary sites. Metastatic tumors accounted for 30% of malignant ovarian tumors. A total of 170 cases of metastatic tumors included 117 cases with nongynecologic origin and 53 cases with gynecologic origin. Nongynecologic metastatic tumors were from large intestine (31%), stomach (14%), intrahepatic bile duct (10%), breast (9%), extrahepatic bile duct/gallbladder (7%), appendix (5%), hematologic tumors (3%), others (4%), and unknown primary site (16%). Metastatic gynecologic tumors were from cervix (53%), corpus (34%), fallopian tube (11%), and gestational trophoblastic disease (2%). The proportion of metastatic tumors to malignant ovarian tumors in northern Thailand was comparable to those of the Western or Japanese studies. However, the distribution of the primary sites was different and was correlated with the cancer incidence in Thai women. The majority of mucin-producing adenocarcinomas involving the ovaries were metastatic tumors.

Outcomes of abandoned radical hysterectomy in patients with stages IB-IIA cervical cancer found to have positive nodes during the operation.

The objective of this study was to evaluate the outcomes of stages IB-IIA cervical cancer patients whose radical hysterectomy (RH) was abandoned for positive pelvic nodes detected during the operation compared with those found to have positive nodes after the operation. Among 242 patients with planned RH and pelvic lymphadenectomy (RHPL) for stages IB-IIA cervical cancer, 23 (9.5%) had grossly positive nodes. RH was abandoned, and complete pelvic lymphadenectomy was performed. Of these 23 patients, 22 received adjuvant chemoradiation, and the remaining 1 received adjuvant radiation. Four patients with positive para-aortic nodes were additionally treated with extended-field irradiation. When compared with 35 patients whose positive nodes were detected after the operation, there were significant differences regarding number of positive nodes and number of patients receiving extended-field irradiation. Complications in both groups were not significantly different, but the 2-year disease-free survival was significantly lower in the abandoned RH group compared with that of the RHPL group (58.5% versus 93.5%, P= 0.01). In conclusion, the survival of stages IB-IIA cervical cancer patients whose RH was abandoned for grossly positive pelvic nodes was significantly worse than that of patients whose node metastasis was identified after the operation. This is because the abandoned RH group had worse prognostic factors.

Prenatal sonographic delineation of the complex cardiac anatomy of thoraco-omphalopagus twins.

We describe a case of thoraco-omphalopagus twins diagnosed at 18 weeks' gestation. Each twin had a separate set of anatomical structures except for a shared heart, upper liver and anterior chest wall. The heart was located at the center of the juncture, and consisted of three atria and two ventricles, each twin having a single ventricle and right atrium but a fused left atrium. A set of great arteries originated from each single ventricle and there was juxtaposition of the aortic and pulmonary valves. One twin had transposition of the great arteries, whereas in the other these were normal in configuration. There was only one umbilical cord which contained four vessels (two arteries and two veins). This case report demonstrates the possibility of using fetal echocardiography to delineate the cardiovascular complex of the conjoined heart.

Well-Differentiated villoglandular adenocarcinoma of the uterine cervix: a report of 15 cases including two with lymph node metastasis.

Well-differentiated villoglandular adenocarcinoma is a recently described subtype of cervical adenocarcinoma. The tumor of this type is reported to have distinct clinicopathologic features and excellent prognosis. However, lymph node metastases of this tumor have been described in few reports. Fifteen cases of well-differentiated villoglandular adenocarcinoma treated at Maharaj Nakorn Chiang Mai Hospital were retrospectively reviewed for both clinical and histopathological features. All patients underwent radical hysterectomy with pelvic lymphadenectomy. In the cases with lymph node metastasis, adjuvant radiation therapy was also given. The patients ranged in age from 22 to 53 years (mean, 39.3). Fourteen patients were FIGO stage IB and one was stage IIA. All patients had exophytic friable cervical masses. Tumor size known in 14 cases ranged from 1.5 to 4 cm (mean, 2.3). Eleven tumors (73.3%) were confined to the inner third of the cervical stroma with 9 of these (60%) showing only superficial invasion (depth < or = 3 mm). The tumors invaded deeply to the middle third in 3 cases (20.0%), and to the outer third in one (6.7%). Lymphatic invasion was observed in 3 cases, two of them had pelvic lymph node metastasis. Both patients had tumors involving deeper than the inner third of the cervical wall. The follow-up duration ranged from 21 to 144 months (mean, 67.5). Four of thirteen cases without nodal metastasis were lost to follow-up 36 to 59 months after surgery. All patients showed no evidence of disease at the last visit. Presence of lymphatic invasion and deep stromal involvement appeared to be the risk factors for lymph node metastasis of well-differentiated villoglandular adenocarcinoma.

Loop electrosurgical excision procedure (LEEP) at Maharaj Nakorn Chiang Mai Hospital: problems in pathologic evaluation.

BACKGROUND: Loop electrosurgical excision procedure (LEEP) is widely used in diagnosis and management of cervical lesions. Difficulties in histopathologic evaluation of LEEP specimens, particularly for the margin status, have been reported to be a significant disadvantage of the procedure. METHOD: The histologic slides of the specimens from 163 patients who underwent LEEP at Maharaj Nakorn Chiang Mai Hospital from August 1995 to November 1997 were retrospectively reviewed for the degree of thermal artefact and the margin status. Follow-up data after a 6-month-period were correlated with the margin status. RESULTS: Thermal artefact was present in all cases (mild 51.5%, moderate 36.2%, and severe 12.3%). In only one case, histologic diagnosis of the lesion was not possible due to severe thermal artefact. Nine cases (5.5%) had non-evaluable margins due to either thermal artefact (7 cases) or improper orientation of fragmented tissue (2 cases). Of 90 cases with subsequent surgical specimens, residual diseases were present in 4 of 21 (19.0%) with negative LEEP margins, in 31 of 64 (48.4%) with positive margins, and in 4 of 5 (80.0%) with non-evaluable margins. CONCLUSIONS: Pathologic evaluation of the specimens from LEEP was limited in only a minority of cases. Thermal artefact was not a critical disadvantage of LEEP. The positive or negative margin status was correlated with the risk of residual disease.

Cytology of small-cell carcinoma of the uterine cervix in serous effusion: a report on two cases.

Cytologic features of 2 cases of small-cell carcinoma of the uterine cervix in the body fluid are described. Case 1 was a 34-yr-old woman with a stage IIA cervical tumor. Pleural effusion developed 6 mo after initial diagnosis. Case 2 was a 38-yr-old woman with a stage IB tumor. Ascites was detected 11 mo after hysterectomy. Histologically, both cervical tumors were indistinguishable from small-cell carcinoma of oat-cell type in the lungs or other sites. Cytologically, the tumor cells in the pleural effusion of case 1 had characteristic features of small-cell carcinoma, including nuclear molding. However, almost all tumor cells in the ascites of case 2 showed a single-cell pattern mimicking malignant lymphoma. Mitotic figures and karyorrhetic bodies were occasionally seen. Nuclear molding was rarely identified. Small-cell carcinoma should be included in the differential diagnosis of malignant effusions containing lymphoma-like cells.

Prenatal diagnosis of VACTERL association: a case report.

A prenatal diagnosis of VACTERL association, a combination of vertebral (V), anal (A), cardiac (C), tracheoesophageal (TE), renal (R) and limb (L) anomalies was made at 30 weeks of gestation, based on the sonographic demonstration of vertebral defects, bilateral renal agenesis, and left lower limb defects. Additionally, severe oligohydramnios and fetal growth restriction were also documented. After proper counseling, elective termination of pregnancy was done, resulting in a stillborn infant with multiple malformations compatible with the VACTERL association. The postnatal X-ray and autopsy revealed verterbral defects, anorectal atresia with undetermined sex, cardiac defect of ventricular septal defect, tracheal agenesis with distal atresia of esophagus, bilateral renal agenesis, and limbs defects. The chromosomal study revealed normal, 46,XY. This report emphasizes the important role of prenatal ultrasound in the diagnosis and management of this disorder.

Prenatal ultrasound of regional akinesia with Pena-Shokier phenotype.

This report describes sonographic features of the Pena-Shokeir phenotype secondary to regional akinesia at 28 weeks of gestation with maternal perception of good fetal movement. The diagnosis was based on the findings of no fetal activity in some parts of the body (upper limbs, a part of the face, and thorax), with deformation sequence of fixed flexion at wrist, elbow, and shoulder joints, fixed open mouth, fetal growth restriction, lung hypoplasia, polyhydramnios and normal chromosome study. Surprisingly, fetal akinesia involved only the upper limbs, a part of the face, and chest, whereas the lower limbs were completely normal in both morphology and activity. With vibroacoustic stimulation, the response of fetal heart rate acceleration, gross body movement including fetal head, spine and lower limb movement were observed, but the movement of the upper limbs and fetal breathing were completely absent. Spontaneous labour and delivery occurred at 29 weeks' gestation. Postnatal autopsy confirmed the prenatal findings. In conclusion, prenatal sonography plays an important role not only in detecting Pena-Shokeir phenotype but also in providing the detailed pattern of fetal akinesia. Finally, this case reaffirms the concept that function is an integral part of normal development.

Childhood Churg-Strauss syndrome.

Churg-Strauss syndrome or allergic granulomatosis and angiitis is a vasculitis that is found in adults, but is extremely rare in children. We describe a 14-year-old boy who presented with prolonged fever, weight loss, sinusitis, myalgia and arthralgia, testicular pain, pulmonary infiltrations, pericardial effusion, peripheral neuropathy, and eosinophilia. Muscle biopsy showed necrotizing arteritis with eosinophil infiltration. His clinical course was complicated by several seizures secondary to cerebral vasculitis and severe asthma, resulting in death. The clinical features and outcomes of childhood Churg-Strauss syndrome are reviewed.

Proliferating (LMP) mucinous tumors of the ovaries with microinvasion: morphologic assessment of 13 cases.

From a consecutive series of 124 proliferating (low malignant potential) mucinous ovarian tumors, 13 FIGO stage Ia tumors exhibited 1 or more microfoci of stromal invasion. The proliferating mucinous tumors with microinvasion were of intestinal (7/88), endocervical-like (4/26), or mixed epithelial differentiation (2/10) and were more likely to show high-grade (10/42) than low-grade (3/82) epithelial proliferative changes. Morphologically, foci of microinvasion were characterized by single or small groups of epithelial cells in the tumor stroma. These cells were accompanied by a sparse lymphocytic infiltrate and stromal desmoplasia in their immediate vicinity and by a greater degree of nuclear anaplasia in the invading cells and in the lining cells of the nearby glands or cysts than was present elsewhere in the tumors. Focal inflammatory and degenerative changes, commonly seen in ovarian mucinous tumors, were discriminated from microinvasive carcinoma by the nature of the inflammatory response, the presence of stromal mucin deposits, and the absence of nuclear anaplasia. Follow-up was available in 10 of 13 cases (mean 7.5 years) and did not identify any adverse clinical consequences for the patients.

Muscular hamartoma of the breast: a rare breast lesion containing smooth muscle.

Hamartoma of the breast is an uncommon entity, usually presenting as a well-demarcated breast mass. Microscopically, the lesion is composed of mammary glandular component, fibrous stroma, adipose tissue, and smooth muscle in variable proportions. Among the variants of breast hamartoma, muscular hamartoma is rare. This lesion should be differentiated from other breast tumors that contain smooth muscle element. We report a breast lesion of a 36-year-old woman diagnosed as a muscular hamartoma in which the muscular component is cellular and some mitotic figures are present. The criteria to distinguish between benign and malignant smooth muscle lesions in the breast, emphasizing mitotic count, are also discussed.

Immunohistochemical characterization of a new monoclonal antibody reactive with dengue virus-infected cells in frozen tissue using immunoperoxidase technique.

This paper presents a novel monoclonal antibody shown to react with cytoplasmic antigens in various dengue infected human frozen organs from autopsy and necropsy specimens. Strong reactivity was found in hematopoietic cells, including immunoblasts, lymphocytes, plasma cells and macrophages of spleen, lymph node, lung, kidney and stomach. Strikingly, strong positivity was demonstrated in cerebral cortex neurones, Purkinje cells, choroid plexus and blood vessels in addition to astrocytes and microglia. Neurotropism of the virus could explain the meningitis, encephalitis, mononeuropathy and polyneuropathy observed by direct toxicity, but noted especially after an activation of mononuclear phagocytes and amplification of the immune response with subsequent vascular inflammation and formation of immune complexes.

Heterotopic gastrointestinal and pancreatic tissue of the tongue: a case report.

Heterotopic gastrointestinal mucosa of the tongue is a rare type of oral choristoma. Associated pancreatic tissue has never been reported to our knowledge. We present the case of an 8-month-old female infant with congenital cleft palate who had a tongue mass. On histologic evaluation the lesion consisted of gastrointestinal and pancreatic tissue. This choristoma may arise from entrapped multipotential endoderm during tongue development.