BACKGROUND Juvenile Hyaline Fibromatosis (JHF) is a rare autosomal recessive connective tissue disorder with distinct clinical and histopathological features. Approximately, 70 cases of JHF have been reported worldwide with only a handful of cases from India. Herein, we report a case of JHF in a middle-aged man whose sole presentation was multiple variably-sized painless nodules over the entire body since childhood with history of repeated surgical excision for the same. Histopathology revealed proliferation of fibroblasts lying in a background of PAS positive amorphous hyaline ground substance. The genetic basis of this disorder was recently mapped, which led to the basis of aberrant synthesis of glycosaminoglycans and disordered collagen metabolism. JHF has a relentlessly progressive course with no specific treatment available till date.
A rare case of placental mesenchymal dysplasia (PMD) in a 26-year-old patient is reported. Ultrasound scan at 17 weeks of gestation showed placenta with multiple cystic spaces and a normal appearing fetus. Following delivery of a term live baby, histological examination of the placenta was suggestive of PMD. The early recognition of this rare condition by characteristic ultrasonographic findings is herein emphasized and hence that PMD is distinguished from molar pregnancy.
An eleven-year-old female child presented with complaints of head ache and vomiting for 1 month. Routine blood examination was within normal limits. MRI shows a heterogenous enhancing lesion of mixed signal density with cystic and solid component involving right frontal lobe extending into the perisylvian region suggestive of PNET/Atypical Teratoid/Rhabdoid Tumour. She underwent excision biopsy from right frontal region
BACKGROUNDSoft tissue sarcomas (STS) account for less than 1% of all malignant tumours but present a significant diagnostic and therapeutic challenge since there are more than 50 histological subtypes. 1 The clinical course of a sarcoma cannot be predicted by histologic typing alone but must be accompanied by grading and staging. Sarcomas with high metastatic potential can be picked out by histologic grading which is a cheap and simple method. One widely used and most reproducible grading system is French Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) based on a score obtained by evaluating tumour differentiation, mitotic rate and amount of tumour necrosis.2 Histologic grading is the mainstay in predicting prognosis of STS especially in centres where facilities for more sophisticated methods are not available. Hence this study was undertaken to assess the effectiveness of the FNCLCC grading system in the prognostication of STS in our patient population. Aims and Objectives 1. To grade soft tissue sarcomas according to the FNCLCC system. 2. To study the association between grade and survival of soft tissue sarcomas in the patient population.
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