Presacral parachordoma causing intestinal obstruction in a patient with renal failure: Report of a case.

Parachordoma is usually lobulated and pseudoencapsulated, grows slowly, and is only locally invasive. It rarely occurs in the retroperitoneal area. Differential diagnoses include chordoma, extraskeletal myxoid chondrosarcoma, and subcutaneous sacrococcygeal myxopapillary ependymoma. We herein report our experience with a huge presacral parachordoma causing intestinal obstruction. A 48-year-old woman was a hemodialysis-dependent patient with end-stage renal disease. She had been diagnosed with uterine myomas several years prior. The patient presented with signs of intestinal obstruction. A presacral tumor with severe compression on the rectum resulting in rectal stricture was found. A resection of the presacral tumor was difficult owing to its large size and the difficult location of the tumor, tendency for high blood loss, and the patient's poor physiological status secondary to uremia. Resection of the lesion was complicated by intraoperative bleeding and late occurrence of a pelvic abscess, which was successfully treated by computed tomography-guided drainage.

Primary diaphragmatic yolk sac tumor and review of the literature.

Primary yolk sac tumor of the diaphragm in children is very rare, and diagnosis of a diaphragmatic tumor poses challenges to clinical physicians. Here, we report a primary diaphragmatic yolk sac tumor in a 9-month-old girl, together with a review of 4 earlier reported cases in the English literature. Carboplatin-containing regimen successfully decreased the tumor size and a total resection of the tumor was made subsequently. The patient was disease-free 8 months after the completion of treatment.

Ectopic thymic carcinoma in the neck.

Thymic carcinoma is a rare tumor arising from the thymus, which is most commonly located in the anterior mediastinum. We report a 24-year-old woman who presented with a neck tumor. The patient underwent complete resection and the pathology sections showed thymic carcinoma. The patient received adjuvant chemotherapy and radiotherapy. After a 6-month follow-up, the patient is well without any evidence of recurrence.

Ovarian borderline mucinous tumor with stromal microinvasion and hemangiomatous mural nodules.

A 39-year-old female presented with abdominal distension. A right adnexal mass was found on physical examination, which was shown to be cystic on ultrasound. An exploratory laparotomy revealed a right ovarian mass, which was removed and a staging procedure was performed. Histologically, the mass was a borderline ovarian tumor with stromal microinvasion and hemangiomatous mural nodules.

Systemic cat scratch disease.

Systemic cat scratch disease (CSD) is often associated with prolonged fever and microabscesses in the liver and/or spleen. We report a case of systemic CSD with hepatic, splenic and renal involvement in an aboriginal child in Taiwan. A previously healthy 9-year-old girl had an intermittent fever for about 17 days, and complained of abdominal pain, headache and weight loss. Abdominal computed tomography showed multiple tiny hypodense nodular lesions in the spleen and both kidneys. Laparotomy revealed multiple soft, whitish-tan lesions on the surface of the liver and spleen. Histopathologic examination of a biopsy specimen of the spleen showed necrotizing granulomatous inflammation with central necrosis surrounded by epithelioid cells and occasional Langhans' giant cells, strongly suggestive of Bartonella henselae infection. History revealed close contact with a cat. B. henselae DNA was detected by polymerase chain reaction in the tissue specimen, and the single antibody titer against B. henselae was greater than 1:2048. These results confirmed the diagnosis of visceral CSD caused by B. henselae. The patient's symptoms resolved after treatment with rifampin and tetracycline. This case illustrates the need for inclusion of systemic CSD in patients with fever of unknown origin and abdominal pain.

Expression of estrogen receptor-alpha and Ki67 in relation to pathological and molecular features in early-onset infiltrating ductal carcinoma.

Estrogen causes breast cancer by triggering proliferation via an estrogen receptor (ER)-mediated mechanism. However, paradoxically, ER alpha, one of the two known ER subtypes, and the proliferation marker, Ki67, are not usually expressed in the same breast tumor. To explore whether ER alpha-positive tumors and proliferating (Ki67-positive) tumors have different tumorigenic characteristics, we performed an immunohistochemical study on 74 early-onset infiltrating ductal carcinomas of the breast. To test this hypothesis, we examined whether ER alpha-positive and Ki67-positive tumors showed differences in (i) pathological grade, (ii) three indices of tumor grade (tubule formation, nuclear pleomorphism, and mitotic number), and (iii) expression of important proteins implicated in breast tumorigenesis (cyclin D1, ErbB2, ATM, BRCA1, Rb, p53, and p21). The results of the multigenic analysis showed that ER alpha and Ki67 were the only two important markers significantly and independently associated with tumor grade, consistent with the above hypothesis. ER alpha-positive, Ki67-negative tumors frequently displayed a low tumor grade (i.e. being well differentiated), whereas Ki67-positive, ER alpha-negative tumors were more likely to exhibit a high tumor grade. In addition, positive ER alpha expression (46 of 74 cases, 62%) correlated well with positive cyclin D1 expression (p < 0.005), less nuclear pleomorphism (p < 0.001), and a low mitotic count (p < 0.005), whereas positive Ki67 expression (36 of 74 cases, 49%) correlated with reduced BRCA1 expression (p < 0.01) and high mitotic activity (p < 0.01). These findings suggest that the expressions of ER alpha and Ki67 might be involved in distinct pathological and molecular features during breast cancer development.

Expression of p53 protein and Ki-67 antigen in phyllodes tumor of the breast.

BACKGROUND: Phyllodes tumors (PT) of the breast are uncommon, and it is often difficult to predict their clinical behavior from histologic features in individual cases. In addition to routine morphology, the studies of p53 protein and Ki-67 antigen expression in PT may be useful to differentiate benign from malignant tumors. METHODS: Immunohistochemical analyses using monoclonal antibody to label p53 protein and another monoclonal antibody MIB-1 to label Ki-67 antigen were performed on the tissue sections of 63 PT from 56 patients. The percentages of positive staining tumor cells were compared with the tumor gradings and clinical outcomes. RESULTS: According to histologic criteria, this series contained 50 benign and 13 malignant tumors. The p53 protein expression showed a significant difference between benign and malignant lesions. Within the group of benign lesions, 5 out of 50 (10%) tumors had p53 expression > 10%, whereas nine out of 13 (69%) malignant tumors revealed p53 expression > 10% (p < 0.005). The Ki-67 antigen was also well correlated with tumor grading. Eleven out of 13 (85%) malignant tumors but only 8 out of 50 (16%) benign tumors showed Ki-67 antigen increased > 10% (p < 0.005). Three patients progressed from benign to malignant tumors. All the first and recurrent tumors in these 3 patient showed Ki-67 > 10%. CONCLUSIONS: P53 protein and Ki-67 antigen expression are correlated with the histology grading. In tumors with benign morphology but having a Ki-67 antigen > 10%, it is necessary to treat the patient and follow up properly to avoid recurrence and malignant transformation.

Immunohistochemical expression of Wilms' tumor 1 protein in nephroblastoma.

BACKGROUND: Approximately 10% of nephroblastomas (Wilms' tumors) carry mutations in the Wilms' tumor 1 (WT1) gene. Recently, a WT1 antibody raised against N-terminal 1-181 amino acids of human WT1 became commercially available for immunohistochemical use on paraffin-embedded tissue. The aim of this study was to investigate the diagnostic and prognostic value of WT1 N-terminal antibody in nephroblastomas. METHODS: Twenty-five patients with nephroblastoma were studied. Four clear cell sarcomas of the kidney (CCSK) and 15 neuroblastomas were included for comparative study. WT1 immunostaining was performed on paraffin material using the WT1(6F-H2) antibody. The patients were staged according to the National Wilms' Tumor Study (NWTS) staging system. RESULTS: Eleven tumors (44%) showed blastemal nuclear staining with or without epithelial nuclear staining. Three of the 13 low-stage tumors (stages I and II) showed WT1 blastemal nuclear staining, while 8 of the 12 high stage (stage III and IV) tumors revealed blastemal nuclear staining. The blastemal nuclear expressions of WT1 were statistically significantly correlated with clinical stage (p = 0.036). All the neuroblastomas and CCSK showed no nuclear immunoreactivity. CONCLUSIONS: The presence of WT1 nuclear immunoreactivity may be helpful to distinguish blastemal predominant nephroblastomas from CCSK and neuroblastomas.

Sentinel lymph node mapping with emulsion of activated carbon particles in patients with pre-mastectomy diagnosis of intraductal carcinoma of the breast.

BACKGROUND: The most common tracers used for lymphatic mapping in sentinel lymph node dissection (SLND) are blue dye and radio-colloid. The former is associated with hypersensitivity, and the latter is not available in some institutions. It is still unclear as to which subsets of patients benefit most from SLND. In this study, we tried to evaluate the usefulness of activated carbon in SLND in the early stages of breast cancer. METHODS: Patients with palpable lesions diagnosed as ductal carcinoma in situ (DCIS) or intraductal carcinoma with micro-invasion (DCMI) from their core-needle biopsy specimens were eligible for the study. A 0.4-0.6 cc emulsion of activated carbon particles (ACP) was injected peri-lesionally or subdermally, 5 minutes before modified radical mastectomy. When the axillary compartment was entered, the black-stained sentinel nodes (SLNs) were dissected and examined with H&E stain by frozen section. The extension of subsequent axillary lymph node dissection (ALND) was determined by the status of the sentinel nodes. RESULTS: Twenty-eight patients were diagnosed as DCIS and 10 as DCMI initially. The SLNs were successfully localized in 33 patients (86.8%), with an average of 2.4 SLNs dissected. The SLNs were positive for metastasis in three patients, and the non-sentinel axillary lymph nodes (ALNs) were also positive in one of them. The final diagnosis of these patients turned out to be infiltrating ductal carcinoma. The SLNs were negative for metastasis in 30 patients, and all the ALNs of these patients were also negative. Among these 33 patients, the final diagnosis was up-graded in nine (27.3%), including the three patients with positive SLNs. The SLNs were not identified in five patients, and the ALNs were positive for metastasis in one of them. CONCLUSIONS: ACP is an acceptable tracer for lymphatic mapping in SLND. For patients with palpable, biopsy-proven DCIS or DCMI, SLND can be used to select patients for ALND.

Distinguishing multiple rice body formation in chronic subacromial-subdeltoid bursitis from synovial chondromatosis.

Multiple rice body formation is a complication of chronic bursitis. Although it resembles synovial chondromatosis clinically and on imaging, the literature suggests that analysis of radiographic and MR appearances should allow discrimination. We report the imaging findings in a 41-year-old man presenting with rice body formation in chronic subacromial-subdeltoid bursitis. We found that the signal intensity of the rice bodies is helpful in making the diagnosis.

Primary synovial osteochondromatosis of the first metatarsophalangeal joint.

Synovial osteochondromatosis is an uncommon disorder, generally seen in large joints such as the knee, elbow, shoulder, hip and ankle. We report a 35-year-old man with synovial osteochondromatosis of the metatarsophalangeal joint of the great toe. Despite the rarity of the location, the radiographic appearance ought to suggest the diagnosis. Histological confirmation is required, since malignancy cannot be totally ruled out clinically and radiographically.