CD44 loss in gastric stromal tumors as a prognostic marker.

BACKGROUND: The adhesion molecule CD44 (CD44s; CD44H) and its isoforms (CD44v3-6 and v9) are preferentially expressed by different cell types. These transmembrane glycoproteins are involved in cell-cell and cell-matrix interactions and in cell trafficking and, thus, may play a role in tumor metastasis and/or local invasion. The expression pattern of CD44s and variant isoforms, particularly CD44v6 and CD44v9, of some neoplasms, including soft tissue tumors, correlates with clinical course and outcome. The clinical behavior of gastrointestinal stromal tumors (GIST) is site specific; however, other reliable predictors of clinical outcome have not been identified. Thus, the prognostic value of CD44s and isoform expression in GIST were evaluated by immunohistochemistry of tissue microarrays. DESIGN: Paraffin-embedded formalin-fixed tissue cores (129: 103 GIST and 26 normal stomach smooth muscle) from 33 patients with clinical outcome data were collected and used for the construction of the tissue microarrays. One to five tissue cores from each patient specimen were evaluated (mean = 3 tissue cores/patient). Array slides were stained with anti-CD44s (CD44H) and with antibodies to v3, v4, v5, v6, and v9 isomers. CD44s and isoform expression and staining intensity were scored semiquantitatively without knowledge of patient identity or outcome: 0 = no; 1 = weak; 2 = moderate; 3 = moderate to strong; 4 = strong. The scores of multiple cores from the same GIST were averaged; the nonneoplastic smooth muscle was similarly graded. CD44s and isoform expression and intensity were compared with outcome. RESULTS: The 33 patients with gastric GIST, 0.8 to 30 cm in size, were followed for 1 to 111 months with a median follow-up of 7 months (mean 17.5 months). The overall median survival was 25 months. Nine of the 33 (27%) patients had metastases, 9 (27%) had recurrent disease, and 9 (27%) died of disease (9-111 months; mean 39 months; median 23 months). All 18 patients with GIST CD44s expression > 2+ were alive at last follow-up (1-62 months; median 3.5 months; mean 11 months). More than half (53%) of patients with GIST CD44s expression < or = 2+ died (9-111 months; median 23 months; mean 38 months); the median follow-up of the surviving patients with CD44 expression < or = 2 was 5 months (2-22 months; mean 6.5 months; log rank P = 0.07). The majority of tumors were variably positive CD44v3 and CD44v4, but there was minimal staining (number of cases and/or expression level) with antibodies directed against the v5, v6, and v9 isomers. CONCLUSION: These preliminary results suggest that although gastric GISTs variably express CD44s and variants, only the expression of CD44s correlates with clinical outcome with loss of CD44s positivity correlating with poor clinical outcome.

Ramichloridium mackenziei brain abscess: report of two cases and review of the literature.

We report two cases of brain abscesses caused by Ramichloridium mackenziei, a neurotropic dematiaceous fungus that seems to be geographically restricted to the Middle East. One of the patients had chronic myelomonocytic leukemia but did not receive any chemotherapeutic agents. The other patient was a normal host. Both cases had a fatal outcome despite aggressive antifungal therapy and surgical intervention. Herein, we review all previously described cases in the literature, and discuss the epidemiology, mycology and histopathology of this life-threatening organism.

Polypoid endocervical stromal sarcoma with heterologous elements. Report of a case with review of the literature.

A 44 year old woman presented with six recurrent endocervical polyps within the span of 28 months. The last two of these polyps exhibited sarcomatous changes within the endocervical stroma with heterologous cartilagenous elements. Abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. No chemotherapy or radiotherapy was administered. The patient is alive and free of recurrent disease 9 years following surgery.

Adenoid cystic carcinoma of the trachea mimicking asthma.

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The spectrum of breast diseases in Saudi Arab females: A 26 year pathological survey at Dhahran Health Center.

In an attempt to delineate the spectrum of breast diseases in Saudi Arab females, we carried out a retrospective study of all cases of breast biopsies and mastectomies accessioned in the files of surgical pathology in our laboratory for 26 years (1967-1992). A total of 915 cases were collected. Fibroadenoma was the most common lesion encountered (30.7%), followed by fibrocystic condition (21.1%), carcinoma (14.9%), acute mastitis (7.2%), duct ectasia (4.9%), lactational adenoma (4.8%), intraductal papilloma (2.6%), galactocele (2.4%) and several less frequent lesions. Pathological conditions associated with lactation such as acute mastitis, abscess, granulomatous mastitis, galactocele and lactational adenomas constituted 16.2% of the cases in this series. This high frequency is related to the high fertility rate among Saudi Arab females. The mean age of Saudi Arab females with ductal carcinoma was 47.1 years as compared to 54 years in Western countries. Many patients presented with a large size tumor, skin and/or nipple involvement, as well as a high frequency (61.7%) of axillary nodal metastases in those who underwent axillary nodal dissection. The high frequency of fibroadenoma could be related to the large number of young females in our population. A great increase in the number of cases in the last five years has been observed. This could be related to more awareness among Saudi Arab females of their health problems and the expansion of our medical services.

Aggressive angiomyxoma of the vagina.

Aggressive angiomyxoma is a recently described pathological condition which affects mainly the soft tissues of the pelvis and perineum in women. It is neither encapsulated nor circumscribed and has a tendency for local recurrence. We report a 50-year-old woman who presented with a mass arising within the vaginal wall and extending to the perineum which appeared cystic on ultrasound. The mass was excised and there is no evidence of recurrence 40 months after surgery.

Hydatidosis in Jordan: an epidemiological study of 306 cases.

Of the 306 subjects studied, who had had hydatid cysts (Echinococcus granulosus) surgically excised in Jordan, 185 (60%) were female and 121 (40%) male. The liver was the most affected organ (57.8%), followed by the lung (26.4%). The kidneys, spleen, brain, soft tissues of neck, appendix and mesentery were less frequent sites. Multiple-organ infection was seen in 23 patients (7.5%). Over a third of the cases (35.8%) was aged < 21 years and the commonest occupation was housewife, followed by school student. The epidemiology of hydatidosis transmission in Jordan is discussed.

Struma ovarii with pseudo-Meigs' syndrome: report of a case and review of the literature.

A case of struma ovarii with ascites and hydrothorax is reported. This unusual rare association is labeled as pseudo-Meigs' syndrome to differentiate it from conventional Meigs' syndrome in which the ovarian tumor is a fibroma. We discuss the diagnostic problems of this condition, and present a brief review of the reported cases in the literature.

Sudden unexpected death due to papillary fibroma of the aortic valve. Report of a case and review of the literature.

The case of the sudden unexpected death of a 21-year-old man due to embolization of segments of an aortic valve papillary fibroma to the left main and anterior descending coronary artery is presented. The literature regarding cardiac papillary fibroma is reviewed with particular reference to those cases associated with sudden death.

Diagnostic value of cervical lymph node biopsy: a pathological study of 596 cases.

During a 10-year period (1976-1985), a total of 596 cervical lymph node biopsies for diagnostic purposes were performed at our institution. Thirty-five percent of these nodes were involved with malignant lymphoma, and 20.5% showed metastatic deposits. Nondiagnostic reactive hyperplastic changes were noted in 23% of the cases. Several other inflammatory and neoplastic conditions were encountered. Comparison of our data with several series from different countries with review of relevant literature are presented.

Schistosomiasis of the spinal cord in a Yemeni boy. A report of a case with brief review of literature.

A 13-year old Yemeni boy who presented with flaccid paraplegia was thought to have a spinal cord tumor on myelography. Microscopic examination of surgically excised material revealed bilharzial granulomas. Brief review of relevant literature on schistosomiasis of the spinal cord, with particular reference to the Middle East, is presented.

Intramuscular myxoma.

Myxomas of the skeletal muscle are uncommon mesenchymal tumours. About one hundred such tumours have been reported in the world literature so far. In the present paper, we report a case of myxoma which occurred in the thigh muscles, with brief review of pertinent literature.

Sudden, unexpected death due to cardiac fibroma. Report of a case and review of the literature.

A case of cardiac fibroma in a 58-year-old woman with a history of atrioventricular block is described. The patient died suddenly at home 6 days following the insertion of a pacemaker. Review of the pertinent literature is presented.

Aneurysmal bone cyst of the ilium associated with pregnancy.

A 19-yr-old primigravida presented with a back mass which was found to originate from the right ilium and was associated with oblique fetal lie. Elective caesarean section and excision of the mass were done. Histologically, the lesion proved to be an aneurysmal bone cyst. Review of bone tumors associated with pregnancy is presented, with discussion of the problems related to diagnosis of this condition.

Malignant neoplasms in Jordanian children.

Three hundred and seventy-three cases of malignant disease in children were diagnosed in Jordan during the 5-year period 1975-79. Lymphomas (28.7%) were the most frequently encountered tumours, followed by leukaemias (19.6%) and brain tumours (16.1%). One third of the children with lymphoma had Hodgkin's disease. Burkitt's lymphoma accounted for another one third, and lymphocytic lymphoma the remaining third. Abdominal involvement was frequently encountered in children with non-Hodgkin's lymphoma. Our data are similar to those reported from the neighbouring Arab countries, Iraq, Saudi Arabia, Kuwait and Egypt, and contrast with the data available from developed countries in which leukaemias and brain tumours are more frequent than lymphomas.

Burkitt's lymphoma in Jordanian children: epidemiological and clinical study.

Twenty-four children with a diagnosis of Burkitt's lymphoma were seen at Jordan University Hospital during a 6 year period (1978-1983). Their median age was 4 years and the male to female ratio was 3:1. A space clustering of cases was evident. Abdominal mass was the commonest presentation being observed in 21 patients, while only two patients presented with jaw masses. Sixteen patients had advanced disease (stages C and D). Chemotherapy was the basic form of therapy given. Twelve children (50%) survived. Advanced stage and poor nutritional status at diagnosis correlated with poor outcome. Our data indicate that Burkitt's lymphoma in Jordanian children presents an intermediate type that shares some similarities with both the endemic (African) and non-endemic types.

Idiopathic subcutaneous and laryngeal fibrosis.

A 40-year-old totally deaf male presented with submucosal masses in the subglottic space and multiple subcutaneous nodules in the eyelids, scrotum and thighs. Biopsies of these masses revealed dense fibrosis associated with non-specific chronic inflammation. Although the findings were clinically similar to those of neurofibromatosis, amyloidosis, multifocal fibrosclerosis with cutaneous nodules, and familial multicentric fibromatosis, they did not fit completely with any of the above-mentioned conditions. It is possible that this case represents a peculiar entity of its own.