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.

Therapeutic effect of vincristine, adriamycin and prednisolone (VAP) in angioimmunoblastic lymphadenopathy (AIL).

Five patients, three males and two females, with angioimmunoblastic lymphadenopathy (AIL) are described. The two who received steroids had no response and died 2 and 6 months later. The three patients who received vincristine, adriamycin and prednisolone (VAP) went into remission early and are still in remission with a follow-up of 22-35 months. It is concluded that VAP is effective therapy in AIL.

Breast diseases in Jordanian females: a study of 1000 cases.

Pathological data on 1000 breast lesions obtained from Jordanian females were analyzed and compared to data available from other countries or ethnic groups. Ductal carcinoma was the most frequently encountered lesion followed by fibrocystic disease (mammary dysplasia), fibroadenoma and mastitis. The mean age of Jordanian females with ductal carcinoma was 44.5 years, and many patients presented with advanced stage of the disease as evidenced by the high frequency (74.6%) of nodal metastases in the patients who had axillary lymphadenectomy. The frequencies of medullary and mucinous (colloid) carcinoma were not greatly different from those in other countries, but lobular carcinoma had a substantially low rate of occurrence. Many patients with lactation-associated lesions such as mastitis, galactocele and lactating adenomas were noted, which is attributed to the high fertility rate in Jordan.

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.

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.

Craniofacial duplication (diprosopus): report of a case with a review of the literature.

A case of craniofacial duplication (diprosopus) is presented. Details on this rare form of conjoined twins are described, and the proposed theories of its embryogenesis are discussed with brief review of the pertinent literature.

Immature teratoma with gliomatosis peritonei associated with pregnancy.

Immature teratoma is a malignant form of teratoma that contains immature structures resembling those of the embryo. It is rare and accounts for less than 1% of all ovarian cancers. It occurs most frequently in young women but it is very rarely reported in association with pregnancy.

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.

Rickets as an unusual initial presentation of abetalipoproteinemia and hypobetalipoproteinemia.

OBJECTIVE: Description of rickets as an unexpected initial manifestation in two children with abetalipoproteinemia and hypobetalipoproteinemia, and elucidation of its pathophysiology in these conditions. METHODOLOGY: Two infants aged two and six months with abetalipoproteinemia and hypobetalipoproteinemia respectively had clinical rickets at presentation, confirmed radiologically and biochemically. Vitamin D intake and serum levels were measured and other causes of rickets were looked for. RESULTS: Vitamin D intake and laboratory studies levels were suggestive of rickets due to calcium deficiency instead of vitamin D deficiency. Healing of rickets occurred with dietary treatment of the malabsorption, without any dietary calcium or significant vitamin D supplementation. CONCLUSION: Steatorrhea-induced calcium malabsorption seems to be the most likely cause of rickets in this entity.

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.

Polypoid lymphangioma of the tonsil: report of two cases and review of the literature.

Benign tumours of the tonsils are uncommon. Among them, lymphangiomas are rare, and only six well-documented cases have been previously reported. We present two additional cases with review of the literature.

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.

Adenoid cystic carcinoma of the trachea mimicking asthma.

Full text is available as a scanned copy of the original print version.

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.

Microbial contamination of the drinking water distribution system and its impact on human health in Khan Yunis Governorate, Gaza Strip: seven years of monitoring (2000-2006).

OBJECTIVE: To assess total and faecal coliform contamination in water wells and distribution networks over the past 7 years, and their association with human health in Khan Yunis Governorate, Gaza Strip. STUDY DESIGN: Historical data and interview questionnaire. METHODS: Data were obtained from the Palestinian Ministry of Health on total and faecal coliform contamination in water wells and distribution networks, and on the incidence of water-related diseases in Khan Yunis Governorate. An interview questionnaire was conducted with 210 residents of Khan Yunis Governorate. RESULTS: Total and faecal coliform contamination exceeded the World Health Organization's limit for water wells and networks. However, the contamination percentages were higher in networks than in wells. Diarrhoeal diseases were strongly correlated with faecal coliform contamination in water networks (r=0.98). This is consistent with the finding that diarrhoeal diseases were the most common self-reported diseases among the interviewees. Such diseases were more prevalent among subjects who drank municipal water than subjects who drank desalinated or home-filtered water (odds ratio=2.03). Intermittent water supply, insufficient chlorination and sewage flooding seem to be associated with self-reported diseases. Residents in the Gaza Strip have a good level of knowledge about drinking water contamination, and this is reflected in good practice. CONCLUSIONS: Water quality has deteriorated in the Gaza Strip, and this may contribute to the prevalence of water-related diseases. Self-reported diseases among interviewees in Khan Yunis Governorate were associated with source of drinking water, intermittent water supply, insufficient chlorination, sewage flooding and age of water networks.

Renal amyloidosis in Jordan.

Twenty cases of renal amyloidosis were observed among 182 patients who underwent kidney biopsy at Jordan University Hospital (JUH) during the period 1979-1983. Eight cases were classified as primary amyloidosis while the remaining 12 were secondary to various underlying diseases, the most significant one was familial Mediterranean fever (FMF). Comparison of incidence of renal amyloidosis in kidney biopsy series from neighbouring countries is 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.