Primitive neuroectodermal tumors (
PNETs) are malignant proliferations of small, undifferentiated neuroectodermal
cells occurring mainly in
children and share the same reciprocal translocation between
chromosomes 11 and 22 and the same patterns of biochemical and
oncogene expression as osseus and extraosseus
Ewing's sarcoma. Some
PNETs occur in the
brain, while others (the peripheral
PNETs) occur in sites outside the
brain, such as in the
extremities,
pelvis and the
chest wall. They mostly originate in the
chest,
pelvis and retroperitoneum; in rare cases, occurrence in the
head and
neck area has also been seen. We present a rare case of
primitive neuroectodermal tumor arising in the
prostate gland in a 25-year-old
male. To the best of our
knowledge, this is the first
documentation of a primary
peripheral primitive neuroectodermal tumor in the
prostate gland of any
patient from
India and rarely reported in English
literature.