Craniorachischisis Totalis with Congenital Diaphragmatic Hernia-A Rare Presentation of Fryns Syndrome.

Fryns syndrome is a multiple congenital anomaly syndrome with an autosomal recessive inheritance. Here we describe the autopsy case findings of a 19-week male fetus, born out of a consanguineous marriage. The dissection revealed left-sided diaphragmatic hernia, resulting in pulmonary hypoplasia and shift of heart to the right side. In addition, anencephaly and spina bifida throughout the vertebral column were observed. All six criteria for Fryns syndrome were met. Such a presentation of Fryns syndrome associated with Craniorachischisis Totalis has not been reported so far. We have also tabulated the overlapping features of some multiple congenital anomaly syndromes that need to be distinguished at autopsy for an accurate diagnosis.

Oral hyaline ring granuloma: a case report.

An unusual granulomatous inflammatory lesion characterised by the presence of ring shaped hyaline bodies accompanied by foreign body giant cell reaction has been mentioned in the literature. A lack of agreement exists on the nature of these hyaline rings. Opinions ranging from their being hyaline degenerative blood vessels to remains of leguminous cells have been postulated. Although the nature and origin of these bodies is uncertain, there is now considerable evidence that they represent, at least in part, vegetable material, especially pulses, that have been implanted in the tissues. An inflammatory oral lesion in a 17-years-old male patient caused due to traumatic implantation of the vegetable matter (sugar cane) has been reported in this paper. Based on the histological finding of a granulation tissue surrounding a hyaline ring the lesion was diagnosed as hyaline ring granuloma.

Perlecan (basement membrane heparan sulfate proteoglycan) and its role in oral malignancies: an overview.

Perlecan means pearl-like structures. Perlecan is a large proteoglycan (400-500 kDa) present in virtually all vascularized tissues with a distribution that is primarily confined to basement membranes including those of oral mucosa. It is a basement membrane-type heparan sulfate proteoglycan. Perlecan is synthesized by basal cells and fibroblasts adjacent to the basal lamina . Perlecan is also synthesized by vascular endothelial and smooth muscle cells present in the extracellular matrix. It has been demonstrated in recent years that perlecan is distributed in the stromal space of various pathophysiological conditions. The complex pleiotropy of perlecan suggests that this gene product is involved in several developmental processes, at both early and late stages of embryogenesis, as well as in cancer and diabetes. In the oral cavity, perlecan expression is reported to basal cells in normal mucosa and its expression increases in precancer and cancerous conditions. It is also expressed in various odontogenic tumors such as ameloblastoma, keratocyst odontogenic tumor, and also salivary gland tumors such as adenoid cystic carcinoma, mucoepidermoid carcinoma, etc.

Ovarian tumours: a study of 282 cases.

Histologically 282 ovarian tumours were studied, of these 212 (75.2%) were benign, 8 (2.8%) of borderline malignancy and 62 (21.9%) were malignant. The tumours were classified according to WHO classification. Epithelial tumours were the commonest variety constituting 70.9% of all the ovarian tumours followed by germ cell tumours (21.2%), sex cord stromal tumours (6.7%) and metastatic tumours (0.7%). Among the individual tumours, serous tumours (42.9%) were the commonest, followed by mucinous tumours (25.5%), teratomas (17%), granulosa cell tumours (6.7%), dysgerminomas (2.48%) and endodermal sinus tumours (1.77%). Two cases (0.7%) of endometrioid carcinomas and one case (0.35%) of clear cell carcinoma were encountered. Ovarian tumours were found to occur in the age range of 8 months to 74 years. Peak incidence was between 21 and 40 years age group.