Inactivating mutations in the PTCH gene, a human homologue of the Drosophila segment polarity gene patched, have been identified recently in patients with nevoid basal cell carcinoma syndrome. These patients are predisposed to various neoplasias including basal cell carcinomas and medulloblastomas (MBs). To determine the involvement of PTCH in sporadic MBs, which represent the most frequent mal...

INTRODUCTION To present a case of a child with a desmoplastic fibroma of the vidian canal, compressing the vidian nerve. CASE REPORT A 12-year-old girl with several years' history of right-sided facial pain was referred to our institution. Magnetic resonance imaging and computed tomography scans showed an expansile mass involving the right vidian canal. The patient underwent a complete endosc...

Desmoplastic small round cell tumor (DSCRT) is a rare variant of sarcoma with a highly aggressive behavior. It usually affects abdominal cavity and has a male predominance. Its correct diagnosis and treatment is sophisticated and requires an experienced multidisciplinary team. Hereby we present a 25 yr old man from Kerman Province in 2013 with abdominal mass and ascites who underwent sonography...

Desmoplastic fibroma of bone is a rare myofibroblastic tumor, locally aggressive, benign lesion that histologically resembles a desmoid tumor of the soft-tissue. It was initially described in 1958 by Jaffe, who separated it as a distinct entity from other intraosseous fibrous tumors. This tumor constitutes <1% of bone tumors and about 0.3% of all benign osseous tumors and it usually involves th...

We describe the case of a 14-year-old boy who presented with a large, 17.6-cm retroperitoneal mass, along with multiple metastases, and was diagnosed with desmoplastic small round cell tumor. After initial chemotherapy, he underwent gross total resection with a positive margin. On postoperative radiation planning computed tomography, a 6.8-cm heterogeneous mass was noted in the surgical bed. Gi...

Pancreatic cancer is characterized by its late detection, aggressive growth, intense infiltration into adjacent tissue, early metastasis, resistance to chemo- and radiotherapy and a strong "desmoplastic reaction". The dense stroma surrounding carcinoma cells is composed of fibroblasts, activated stellate cells (myofibroblast-like cells), various inflammatory cells, proliferating vascular struct...

Desmoplastic melanoma (DM) is a rare variant of spindle-cell malignant melanoma. DM is easily misdiagnosed at an early stage because it can be confused with benign entities. Histological analysis, including careful attention to the presence of atypical spindle cells, as well as to lymphocytic aggregates in an abundant fibrotic stroma in the dermis, provides clues for diagnosis. The adjunction o...