Objective: Desmoplastic small round cell tumor (DSRCT) is a rare intraabdominal neoplasm that grows along serosal surfaces and is primarily found in young men. To date, only 16 cases of ovarian DSRCT have been previously reported in women in the English literature, and no large population-based studies on this topic exist.
Case Report: We report the case of a 19-year-old virgo with unremarkable past medical history, initially presented with abdominal fullness. After being treated with the optimal treatment modality (primary and secondary surgical debulking, unique chemotherapy, protocol and adjuvant radiotherapy), the patient has remained without tumor disease for 40 months.
Conclusion: Although the best therapy for patients with DSRCT has yet to be determined, combining complete surgical resection, adjuvant chemotherapy, and radiotherapy is required to prolong survival and to achieve proper quality of life.
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http://dx.doi.org/10.1055/s-0040-1710350 | DOI Listing |
J Cutan Pathol
December 2024
SkinPath Solutions, Smyrna, Georgia, USA.
Capicua transcriptional repressor (CIC)-rearranged sarcoma (CRS) is a rare and recently described tumor that most commonly affects patients between 15 and 30 years of age. It is an undifferentiated round cell malignancy, with a disease defining CIC fusion, with double homeobox 4 (DUX4) being the most common partner. Here, we report a 77-year-old woman who presented with a cutaneous thigh mass with a clinical morphology suggesting Merkel cell carcinoma.
View Article and Find Full Text PDFJ Cytol
November 2024
Department of Pathology, Cytogenetics Unit, Kidwai Memorial Institute of Oncology, Bangalore, Karnataka, India.
Background: The role of fine needle aspiration cytology (FNAC) in the diagnosis of renal malignancies is established and has been getting more precise and important over a period of time. Knowledge of the pathology of uncommon renal neoplasms along with radiological and clinical correlations often aids in correct diagnosis.
Aims: The present study aims to describe the cytomorphological and immunohistochemical findings in the varied spectrum of renal tumors, other than renal cell carcinomas (RCC).
Am J Case Rep
December 2024
Department of Molecular Medicine and Surgery, Center for Molecular Medicine, Karolinska Institute, Stockholm, Sweden.
BACKGROUND Limb-girdle muscular dystrophy recessive 1 (LGMDR1) is an autosomal recessive degenerative muscle disorder characterized by progressive muscular weakness caused by pathogenic variants in the CAPN3 gene. Desmoplastic small round cell tumors (DSRCT) are ultra-rare and aggressive soft tissue sarcomas usually in the abdominal cavity, molecularly characterized by the presence of a EWSR1::WT1 fusion transcript. Mouse models of muscular dystrophy, including LGMDR1, present an increased risk of soft tissue sarcomas.
View Article and Find Full Text PDFEur Burn J
December 2024
Helsinki Burn Centre, Department of Plastic Surgery, Helsinki University Hospital and University of Helsinki, 00029 Helsinki, Finland.
Autologous skin grafts are the gold standard for definitive wound coverage in burn care, but allograft skin grafts are essential for providing temporary coverage in cases of extensive burns. The Helsinki Skin Bank, established in 1995 at the Helsinki Burn Centre, is Finland's only licensed skin bank, serving a population of 5.5 million.
View Article and Find Full Text PDFOphthalmic Plast Reconstr Surg
October 2024
The Operation Eyesight Universal Institute for Eye Cancer, Ophthalmic Pathology Laboratory, LV Prasad Eye Institute, Hyderabad, India.
A 40-year-old woman presented with a mass in her OS for 2 years. Examination revealed a large conjunctival lesion on the nasal bulbar conjunctiva OS and a small upper tarsal conjunctival lesion in the OD. Biopsy OD revealed inflammatory granulation tissue, and OS revealed pseudoepitheliomatous hyperplasia with granulation tissue.
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