Aggressive angiomyxoma in African women: a report of two cases.

BACKGROUND: Aggressive angiomyxoma (AAM) is a rare soft tissue tumour usually of the perineum. There is no report in the surgical literature of a description of AAM in black Africans. OBJECTIVE: To report the first description of AAM in black Africa women in the surgical literature and to highlight the value of special immunostains in the complete characterization these rare tumours. METHODS: Case one was a 38-year-old pre-menopausal woman who presented with a five-year history of a painless mass in the left buttock extending to the left side of the perineum with recent ulceration. Clinical examination revealed a pale and febrile woman with an ulcerated 60 x 40 cm mass distorting the left gluteal region and the left side of the perineum. In case two, a 28-year old woman reported for the assessment of pedunculated mass arising from the right labium major that has been present for four years. Clinical examination revealed a 19 x 15.5 cm well-circumscribed mass in the perineum. The mass was completely covered by thickened hairy skin and attached to the right labium majored by a short thick stalk that measured 5 cm x 7 cm in size. Both tumours were excised via incisions in the perineum. RESULTS: In both cases the histopathology of the surgical specimens was reported as bland hypocellular tumours with spindle and stellate cells that lacked mitotic activity consistent with a diagnosis of an aggressive angiomyxoma. CONCLUSION: The clinical and histopathological features of the tumours described in this report are consistent with a diagnosis of aggressive angiomyxoma. To the best of our knowledge this is the first ever report of AAM in black African women.

Genital human papillomavirus testing by in situ hybridization in liquid atypical cytologic materials and follow-up biopsies.

OBJECTIVE: To describe cases of HPV testing by DNA in situ hybridization performed on atypical cervicovaginal samples collected by a liquidsed method that were negative for HPV DNA on cytology but revealed cervical intraepithelial neoplasia on follow-up biopsies. STUDY DESIGN: Three hundred ninety-five consecutive SurePath atypical squamous cells of undetermined significance (ASC-US) cytologic samples from asymptomatic, reproductive-age women were tested for human papillomaviruses (HPVs) by the in situ hybridization (ISH) method (Ventana Inform HPV Test, Tucson, Arizona, U.S.A). One hundred (25%) cases underwent follow-up colposcopic biopsy within 3 months of cytology. All the tests (cytology, ISH, histology) were independently evaluated without knowledge of the other tests. RESULTS: One hundred twenty-two (33%) cytologic samples were positive for HPVs. Of a total of 100 (HPV positive and negative) follow-up biopsies, 55 were positive for cervical intraepithelial neoplasia (CIN). Fourteen cases of biopsy-proven CIN tested negative for all HPV types in the prior cytologic samples. Retesting of the 14 CIN tissues by ISH was negative in 10, positive for HPV in 2 and inconclusive in 2. CONCLUSION: There is a small but significant (14%) false negative rate with HPV testing by the Ventana ISH method. Clinically suspicious cases should be followed even if an HPV test is negative.

Familial ovarian carcinoma. Clinical nuances.

Familial ovarian carcinoma has been recognized with increased frequency during the past decade commensurate with physician attention to family history. Putative autosomal dominant inheritance of this heterogeneous problem mandates attention to both paternal and maternal lineages. A family with probable paternal transmission of breast/ovarian carcinoma is presented. One family member had findings consonant with papillary serous adenocarcinoma of ovarian origin, which may have arisen directly from extra-ovarian pelvic mesothelium. These and other clinical nuances of familial ovarian carcinoma are discussed in order to aid physicians in understanding the natural history, surveillance, and management of familial ovarian carcinoma.

Intrapericardial giant lymph node hyperplasia.

This report concerns a female patient aged 42 years known to have asymptomatic cardiomegaly for 2 years. She presented 2 weeks before surgery with chest pain radiating to the left arm and face. Angiography revealed a highly vascular mass in the region of the left atrium. Surgery revealed an intrapericardial tumor adherent to both atria. Histologically this proved to be giant lymph node hyperplasia, an unusual example because of its intrapericardial localization.

Constrictive calcific pericarditis following coronary arterial bypass surgery.

A case of postsurgical constrictive calcific pericarditis is reported. The unusual features of this case are the short interval (two months) from surgery to the development of pericarditis and the involvement of the coronary arterial grafts, resulting in tearing of the vessels and the death of the patient during decortication.

Prospective peer review in surgical pathology.

Quality assurance (QA) in surgical pathology has focused primarily on retrospective audits of randomly selected cases. The authors describe an effective method of prospective audit for a selected class of surgical specimens--diagnostic biopsies--and document the benefits, additional staff time required and impact on turnaround time. Additionally, these results were compared with a retrospective review. During a 6-month period, all diagnostic surgical pathology biopsies (n = 2,694, 55% of all cases) were reviewed by a second pathologist before release of the final report. Errors detected were subdivided into four categories: (1) major: errors in diagnosis that could directly affect patient care; (2) diagnostic discrepancies: errors in diagnosis that should not affect patient care; (3) minor: correct diagnosis rendered, but report correction required to add supportive information; (4) clerical: typographical and grammatical errors. Thirty-two major errors were found, involving 1.2% of cases reviewed. This manner of review caused an increase in overall turnaround time from 1.62 days to 1.79 days, and an increase in turnaround time for diagnostic biopsies from 1.44 days to 1.50 days. Time spent in performing prospective peer review averaged 4 hours per day. For comparison, results were included from a retrospective review performed on 480 of the 5,556 cases accessioned in a 6-month period before the institution of prospective quality assurance. This retrospective review revealed eight major errors (1.7%). In conclusion, the prospective peer review of diagnostic biopsies yields sufficient benefits in increased accuracy of diagnostic reports to justify the slight increase in additional work by pathologists.

Cystic adventitial degeneration of the femoral artery: is evacuation and cyst excision worthwhile as a definitive therapy?

A case of cystic adventitial degeneration of the left common femoral artery in a patient with localized left groin pain, normal distal pulses, and normal arteriographic findings is reported. This patient was first treated with evacuation and cyst excision. Recurrence was noted after 20 months, and an excision of the cyst and a segment of the common femoral artery with graft interposition was required. At gross examination, the cyst was unilocular and contained gelatinous material. The cyst appeared to be situated in the tunica adventitia and did not communicate with the vascular lumen. No synovial lining was present. Histologically, it was similar to a ganglion cyst with contents rich in hyaluronic acid. A review of the literature was undertaken to determine the results of treating this lesion. The disease is rare. All senior authors of case reports were contacted to construct follow-up information. A high incidence of recurrence was noted in patients treated by evacuation and cyst excision. We believe that total cyst excision with the involved artery and graft interposition at the femoral site can be done easily, safely, and with virtually no chance for recurrence.

Microsatellite instability and expression of MLH1 and MSH2 in normal and malignant endometrial and ovarian epithelium in hereditary nonpolyposis colorectal cancer family members.

Mismatch repair deficiency is a characteristic molecular finding in hereditary nonpolyposis colorectal cancer (HNPCC), and has been demonstrated in both colorectal cancers and benign adenomas. Endometrial and ovarian cancers are common extracolonic tumors in this syndrome; however, few studies have investigated whether genetic changes occur in histologically normal endometrial and ovarian epithelia from HNPCC family members. If early genetic changes exist, they might be used as molecular markers to detect susceptibility to endometrial and ovarian cancers. In this study, we analyzed microsatellite instability (MSI) and MLH1 and MSH2 immunohistochemical expression in 20 histologically normal epithelia (12 endometrial and 8 ovarian) and 8 cancers (4 endometrial and 4 ovarian) obtained from 20 individuals representing 7 unrelated HNPCC families. While MSI was observed in endometrial (75%) and ovarian (100%) cancers, no case was determined to exhibit MSI in histologically normal epithelia of the endometrium or ovary. Similarly, in immunohistochemical expressions for MLH1 and MSH2, histologically normal epithelia had no genetic changes predisposing to malignancy. In cancer cases, a correlation existed between the expression of MLH1 and MSH2, the presence of germline mutations in the hMLH1 and hMSH2 genes, and the presence of tumor MSI. These data suggest that MSI and MLH1 and MSH2 expression are not useful biomarkers for the early detection of endometrial and ovarian malignancy in cancer-unaffected HNPCC germline mutation carriers. Further studies of other genetic changes in normal and premalignant precursor lesions are needed.

Tubuloreticular inclusions in systemic lupus pneumonitis. Report of a case and review of the literature.

Tubuloreticular inclusions have been described with sufficient frequency in certain tissues of patients with systemic lupus erythematosus (SLE) to make the finding of such inclusions helpful in making a diagnosis of this disorder. The finding of such inclusions in lung biopsy specimens, however, has been distinctly rare. We report herein the ultrastructural findings in the case of a young woman with active systemic LE showing tubuloreticular inclusions within lung and kidney biopsy specimens.

Severe intrauterine herpes simplex disease with placentitis in a newborn of a mother with recurrent genital infection at delivery.

We present a case of fatal herpes simplex type 2 (HSV-2) in a premature infant born to a mother diagnosed with recurrent HSV-2, based on history and HSV serology results. It was clinically evident at delivery, and subsequently confirmed by laboratory studies that the infant was infected before delivery. There was histopathologic evidence of placentitis and chorioamnionitis upon examination of the placenta and fetal membranes. This case illustrates a relatively uncommon complication of recurrent genital herpes at delivery--intrauterine transmission to the fetus from a primary episode during pregnancy.

Familial peritoneal ovarian carcinomatosis: a new clinical entity?

A familial form of ovarian carcinoma is now widely recognized. There are at least several ovarian cancer-prone genotypes, consistent with genetic heterogeneity. Prophylactic oophorectomy has been employed for women who were judged to be at 50% risk for this disease by virtue of their position in the pedigree. However, recent evidence has disclosed that a fraction of such patients who underwent prophylactic oophorectomy and who had ovaries which appeared to be histologically normal at surgical resection, subsequently developed intraabdominal carcinomatosis with histologic findings showing the lesions to be indistinguishable from ovarian carcinoma. Given the embryologic derivatives of the ovary, which comprise gonadal ridges composed of mesodermal cells covered by coelomic epithelium, we postulate that patients with hereditary predisposition to ovarian carcinoma harbor the first germinal hit in both the epithelial cells of the ovary as well as their derivatives in the coelomic mesothelium. These patients may then be inordinately susceptible to carcinogenesis from the second (somatic) hit in these same tissues.

Endometrial histology and bleeding patterns in menopausal women treated with estrogen and continuous or cyclic progestin.

Thirty-six postmenopausal women were randomized to three groups in a double-blind, prospective study. All were treated for three months with conjugated estrogens (Premarin), 0.625 mg daily, and three different doses of a progestin (Provera), 2.5 mg daily, 5 mg daily or 5 mg during the last 12 days of a 28-day cycle. We found that the endometrium was maintained in an inactive phase in 100% of the women given continuous daily progestin but in only 25% of those given cyclic progestin. Bleeding occurred in 100% of subjects given cyclic progestin and in 50% of those given continuous progestin; however, bleeding episodes diminished with time in those on continuous progestin. A hormonal regimen that leads to reduced or absent bleeding and an inactive endometrium is preferable for postmenopausal women if estrogen therapy is to be used for the long term after menopause.

Columnar cells in posthysterectomy vaginal smears.

Columnar cells in posthysterectomy vaginal smears are unusual and rare. Nine such cases are reported here during a 6-yr period. All nine patients were asymptomatic and total hysterectomy with bilateral salpingoophorectomy for gynecological malignancies was performed 8 mo to 25 yr ago. Three patients had local radiation and one had systemic chemotherapy not less than 8 mo before the cytology. The columnar cells showed one of three patterns: 1) long, bipolar cells in sheets, resembling reparative columnar cells (4 cases), 2) goblet-type cells with eccentric nuclei (3 cases), and 3) tight clusters of small round cells (2 cases). No consistent relationship with age, treatment, or background was seen with any of the patterns. Follow-up of all cases by thorough pelvic examination, repeat smears, colposcopy, and biopsy showed no vaginal pathology. Benign mucinous or goblet cell metaplasia in atrophic vaginal epithelium may be the source of some of these cells in the vaginal smears.

Fine-needle aspiration biopsy of mucinous cystic neoplasm of the pancreas: a case study.

This report illustrates the cytologic features of well-differentiated cystic adenocarcinoma of the pancreas obtained by fine-needle aspiration biopsy. It also discusses the differentiation of pseudocyst vs neoplastic cyst by this method.

Ultrastructural studies of cells in body cavity effusions.

Transmission electron microscopic examination of benign (16 cases) and malignant (2 cases) mesothelial cells and metastatic carcinoma (10 cases) was performed. These studies showed long, slender, branching and bushy microvilli with high length-to-diameter ratios to be the most important distinguishing features of the mesothelial cells. Cytoplasmic intermediate filaments were present in all mesothelial cells as well as in carcinoma cells. The mesothelial cells showed an absence of mucin vacuoles, intracellular lumens and luminal tight junctions, which are seen in adenocarcinoma cells. The pinocytotic vesicles were found to be more numerous in the mesothelial cells. Lipid vacuoles, lysosomes, Golgi apparatus and intercellular lumens appeared to be variably present in all mesothelial and carcinoma cells. The methodology is discussed and pertinent literature reviewed.

Exfoliative sputum cytology in pulmonary embolism.

In a prospective, double-blind, randomized study of cytologic changes found in pulmonary infarction, nine roentgenologically proven cases of pulmonary embolism were studied with sputum samples from the 1st to 26th postinfarction days. Maximum atypical cytologic changes were seen during the second and third postinfarction weeks. Specific cytologic features included three-dimensional clusters of glandular cells with enlarged nuclei and macronucleoli: they were malignant-appearing cells except for their inconsistent morphology, fewer numbers, transient appearance and lack of solitary atypical cells. A similar study in a canine model is discussed, and a case of pulmonary embolism with abnormal cytology and corresponding histology is presented.

Cytology of peritoneal fluid from patients on continuous ambulatory peritoneal dialysis.

Peritoneal fluids from 41 patients on continuous ambulatory peritoneal dialysis (CAPD) were examined. The patients were divided into a short-term group (18 patients with CAPD up to one year) and a long-term group (23 patients with CAPD for one to seven years). Peritoneal fluids from a control group, consisting of ten nondialysis patients with ascites, were also examined. The cellular background of the peritoneal fluids and, in particular, the morphology of the mesothelial cells were studied. The following were found to be significantly increased in the CAPD groups: background lymphocytes, mesothelial exfoliation in three-dimensional clusters, mesothelial nuclear size and the number of mesothelial nucleoli. All of these features increased slightly with an increased duration of the dialysis. These findings emphasize that peritoneal dialysis of any duration can induce significantly atypical changes in mesothelial cells.