ABSTRACT
ABSTRACT: Calcifying fibrous tumor (CFT) is a very rare benign fibroblastic tumor featuring a wide anatomical distribution and mimicking various spindle cell tumors. CFT has been documented in soft tissues, gastrointestinal tract (GIT), neck, adrenal glands, and pleura. Within the GIT, it is reported in the stomach, small bowel, large intestine, stomach, esophagus, and appendix. The occurrence of CFTs in the gastrointestinal tract presents a diagnostic dilemma, firstly due to the rarity of the lesion and secondly, due to the occurrence of a variety of other stromal lesions in the gastrointestinal tract with histological features that overlap with CFT. In the jejunum, it is extremely rare. We present one such rare case of jejunal CFT at an unusual age, complicated by jejuno-jejunal intussusception resulting in acute intestinal obstruction.
ABSTRACT
Neuroendocrine tumours (NETs) are a rare type of tumours that arise from the neuroendocrine cells which are distributed throughout the body. Of all the gastrointestinal tumours only 1-2% account for NETs. They have an extremely low incidence of 0.17% arising in the intrahepatic bile duct epithelium. Majority of hepatic NETs occur as a result of metastases from the primary NETs. Most cases of primary hepatic NET (PHNET) present as a solid nodular mass. However, predominantly cystic PHNET is extremely rare which mimics other cystic space-occupying lesions clinically and radiologically as seen in this case.
ABSTRACT
Extrarenal rhabdoid tumour of soft tissue in children is a rare tumour associated with poor prognosis. It is a heterogeneous group of aggressive tumours with distinct histopathological and immunohistochemistry findings. The tumour is characterised by diffuse proliferation of rhabdoid cells with hyaline like inclusion bodies. Defining feature is aberration of INI1/SMARCB1 gene located at chromosome 22q11.2. We report one such rare case in a female infant presenting as left axillary mass.
Subject(s)
Rhabdoid Tumor , Sarcoma , Soft Tissue Neoplasms , Infant , Child , Humans , Female , DNA-Binding Proteins/genetics , Transcription Factors/genetics , SMARCB1 Protein/genetics , Rhabdoid Tumor/diagnosis , Rhabdoid Tumor/pathology , Chromosomal Proteins, Non-Histone/genetics , Immunohistochemistry , Sarcoma/pathology , Soft Tissue Neoplasms/diagnosis , Soft Tissue Neoplasms/pathologyABSTRACT
Chronic sclerosing sialadenitis is associated with the immunoglobulin G4 (IgG4)-related disease (RD) spectrum. IgG4-RD is a newly recognized immunomediated fibroinflammatory condition characterized by several features: a tendency to form tumefactive lesions at multiple sites, lymphoplasmacytic infiltrate, fibrosis and obliterative phlebitis. Often but not always, the serum IgG4 concentrations are also elevated. Immunohistochemistry for IgG4 is helpful to clinch the diagnosis. Here, we describe a case of 65-year-old male with IgG4-related chronic sclerosing sialadenitis of the s ubmandibular gland. We have discussed the histopathological criteria to diagnose this entity.
