Desmoplastic small round cell tumors (DSRCT): overview

Desmoplastic Small-Cell Tumor This is a rare disease affecting mainly young people and male children. Usually, a tumour occurs in the abdominal (more often) or thoracic cavity, affects the omega, gastrointestinal tract, bladder and other organs and systems. It is distinguished from other small round cell tumours by characteristic histology – a cluster of blue small round or ovoid cells in a desmoplastic stroma, which is differentiated by epithelial, mesenchymal, myogenic, neural mitosis.

Symptoms of the disease

The most common symptoms are abdominal pain, bloating, a sense of severity and hepatomegaly. Less common signs are retroperitoneal lymphadenopathy, hydronephrosis, bulobestruction, calcification and thickening of the nodal peritoneal lymph nodes. These symptoms are not typical and do not serve as a basis for diagnosis. Therefore, it is important to consider DSRCT as a possible diagnosis when a young person has nonspecific abdominal symptoms and there are radiological signs of disseminated intraperitoneal malignancy.

Knowing the clinical information and characteristic cytological and histological features of the tumor, pathologists and cytopathologists can make an accurate diagnosis of DSRCT. If problems arise in establishing the correct diagnosis, additional studies can be carried out, such as cytogenetic karyotypic analysis and molecular study.

Treatment

Since a desmoplastic small-cell tumor is a rather rare disease, it can only be diagnosed in special laboratories equipped with the latest technology. That is why, when suspected of cancer, parents take their children to the Israeli Tel Aviv Medical Clinic, where there is the necessary advanced equipment. We use innovative examination techniques and unique, child-friendly treatment methods.

The treatment of the disease is a combination of several types, which includes surgery, radiotherapy, medication, certified drugs. The Tel Aviv Medical Clinic provides a full range of medical services of the highest level at prices appropriate to the state. After treatment, children can undergo specialist-led rehabilitation, which contributes to early recovery. For adults who have experienced such a test as a dangerous child’s illness, we have a support center where professional psychological assistance will be provided.