Posted in Molecular pathology

Conditions associated with DICER- 1 syndrome

DICER-1 SYNDROME IS A Rare Autosomal Dominant cancer syndrome Caused by inactivating germline mutations in genes encoding DICER-1. It is an RNase involved in the generation of mature miRNA.

CONDITIONS ASSOCIATED WITH DICER-1 SYNDROME.

1. PLEUROPULMONARY BLASTOMA:

  • Type I tumors are cyst -like tumors in the lung. They are most common in children aged 2 years and younger and have a good chance of recovery. …
  • Type II tumors are cyst-like with some solid parts. These tumors sometimes spread to the brain or other parts of the body.
  • Type III tumors are solid tumors

2. OVARIAN SERTOLI- LEYDIG CELL TUMOR:

Sertoli-Leydig cell tumor (SLCT) is a rare cancer of the ovaries. The cancer cells produce and release a male sex hormone called testosterone. The exact cause of this tumor is not known

3. CYSTIC NEPHROMA OF KIDNEY:

Cystic nephroma is a rare kidney neoplasm belonging to the entity of cystic tumours. It is a slow-growing tumour, which develops insidiously, sometimes reaching a considerable size. The diagnosis is more often accidental (except for mass syndrome in children). It is a benign tumour that may be treated by partial sparing nephrectomy

4.MULTINODULAR GOITRE:

Multinodular Goiter (MNG) is defined as the special condition of thyroid glands, where there are multiple lumps (nodules) formed.

5. PITUTARY TUMOR:

Pituitary tumors are abnormal growths that develop in your pituitary gland. Some pituitary tumors result in too much of the hormones that regulate important functions of your body. Some pituitary tumors can cause your pituitary gland to produce lower levels of hormones.

6. OCULAR MEDULLOBLASTOMA:

Medulloblastoma is the commonest childhood malignant central nervous system tumour. It occurs in the midline of the cerebellum

7. EMBRYONAL RHABDOMYOSARCMA:

Embryonal rhabdomyosarcoma (ERMS) ERMS usually affects children in their first 5 years of life, but it can occur at older ages as well. ERMS tends to occur in the head and neck area, bladder, vagina, or in or around the prostate and testicles.

8.PINEALOBLASTOMA:

Pineoblastoma is more aggressive than other types of pineal gland tumors. Its fast growth usually causes cerebrospinal fluid (CSF) to build up in the brain. This condition is called hydrocephalus. While pineoblastoma may spread through the CSF in 10% to 20% of cases, most of the time the tumors do not spread to other parts of the body.

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