A gangliocytic paraganglioma is a rare tumour that is typically found in the

duodenum The duodenum is the first section of the small intestine in most higher vertebrates, including mammals, reptiles, and birds. In fish, the divisions of the small intestine are not as clear, and the terms anterior intestine or proximal intestine m ...

and consists of three components: (1) ganglion cells, (2) epithelioid cells (

paraganglioma A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen). When the same type of tumor is found in the adrenal gland, they are referred to as a pheochromocytoma. They ar ...

-like) and, (3) spindle cells (

schwannoma A schwannoma (or neurilemmoma) is a usually benign nerve sheath tumor composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves. Schwannomas are homogeneous tumors, consisting only of Schwann cells. ...

-like).

Symptoms and signs

The most common presentation is

gastrointestinal bleed Gastrointestinal bleeding (GI bleed), also called gastrointestinal hemorrhage (GIB), is all forms of bleeding in the gastrointestinal tract, from the mouth to the rectum. When there is significant blood loss over a short time, symptoms may incl ...

(~45% of cases), followed by

abdominal pain Abdominal pain, also known as a stomach ache, is a symptom Signs and symptoms are the observed or detectable signs, and experienced symptoms of an illness, injury, or condition. A sign for example may be a higher or lower temperature than ...

(~43% of cases) and

anemia Anemia or anaemia (British English) is a blood disorder in which the blood has a reduced ability to carry oxygen due to a lower than normal number of red blood cells, or a reduction in the amount of hemoglobin. When anemia comes on slowly, th ...

(~15% of cases).

Pathology

GP consist of three components (1)

ganglion cells {{stack, A ganglion cell is a cell found in a ganglion. Examples of ganglion cells include: * Retinal ganglion cell (RGC) found in the ganglion cell layer of the retina * Cells that reside in the adrenal medulla, where they are involved in the s ...

, (2) epithelioid cells (neuroendocrine-like), and (3) spindle cells (

-like). The microscopic

differential diagnosis In healthcare, a differential diagnosis (abbreviated DDx) is a method of analysis of a patient's history and physical examination to arrive at the correct diagnosis. It involves distinguishing a particular disease or condition from others that p ...

includes poorly differentiated carcinoma,

neuroendocrine tumour Neuroendocrine tumors (NETs) are neoplasm A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of t ...

and

. GPs may be sporadic or arise in the context

neurofibromatosis type 1 Neurofibromatosis (NF) is a group of three conditions in which tumors grow in the nervous system. The three types are neurofibromatosis type I (NF1), neurofibromatosis type II (NF2), and schwannomatosis. In NF1 symptoms include light brown sp ...

. Image: Gangliocytic paraganglioma - intermed mag.jpg , Intermed. mag. Image: Gangliocytic paraganglioma - 2 - intermed mag.jpg , Intermed. mag. Image: Gangliocytic paraganglioma - very high mag.jpg , Very high mag. Image: Gangliocytic paraganglioma - 2 - very high mag.jpg , Very high mag.

References

{{reflist Digestive system neoplasia

Symptoms and signs

Pathology

See also

References