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obsolete small intestinal delta cell somatostatin producing tumor

Description

Based on the provided context, it appears that you are looking for information about a specific type of tumor related to the small intestine.

The context suggests that there is a rare tumor associated with the delta cells in the pancreas and small intestine, which produces somatostatin. This tumor is known as a somatostatinoma.

Here are some key points about this tumor:

  • Definition: A somatostatinoma is a rare neuroendocrine tumor (NET) that originates from the delta cells of the pancreas or small intestine.
  • Function: The primary role of delta cells is to produce and secrete somatostatin, a potent paracrine inhibitor. In the gastrointestinal tract, somatostatin slows down gastric emptying and pancreatic secretion [4].
  • Symptoms: Somatostatinomas are associated with mild diabetes mellitus (due to inhibition of insulin release), steatorrhoea, and gallstones (due to inhibition of cholecystokinin release) [13].
  • Incidence: Somatostatinomas represent less than 1% of functioning gastrointestinal neuroendocrine neoplasms (NENs) and their estimated incidence is about 1 in 40 million individuals per year [12].

It's worth noting that somatostatinomas are rare tumors, and the information provided in the context suggests that they are often large (>5 cm) at diagnosis.

Additional Characteristics

  • Gallstones
  • Somatostatinoma
  • A rare neuroendocrine tumor originating from delta cells of pancreas or small intestine
  • Mild diabetes mellitus
  • Steatorrhoea

Signs and Symptoms

Based on the provided context, here are the signs and symptoms associated with an obsolete small intestinal delta cell somatostatin-producing tumor:

  • Abdominal pain: This is a common presenting symptom, affecting 70% to 100% of patients (6).
  • Diarrhea: Diarrhea is another frequent symptom, reported in 37% to 73% of patients (6).
  • Gastroesophageal reflux disease (GERD): GERD is also a common symptom, affecting 44% of patients (6).
  • Small bowel obstruction, intussusception, or abdominal mass: These symptoms can occur due to the tumor's growth and metastasis (7).

Additionally, somatostatin-producing tumors can cause a range of systemic symptoms, including:

  • Diabetes mellitus: This is a condition where blood sugar levels become too high, caused by excessive somatostatin secretion (13).
  • Weight loss: Weight loss can occur due to the tumor's effects on digestion and metabolism (14).

It's worth noting that these symptoms may vary depending on the specific location and size of the tumor, as well as individual patient factors.

References:

[6] - Symptoms caused by somatostatin-producing tumors. [7] - Signs and symptoms of small bowel obstruction, intussusception or abdominal mass. [13] - Diabetes caused by excessive somatostatin secretion. [14] - Weight loss due to tumor effects on digestion and metabolism.

Diagnostic Tests

Based on the provided context, it appears that diagnostic tests for a rare and obsolete type of small intestinal delta cell somatostatin-producing tumor are not well-documented. However, I can provide some general information on diagnostic tests for similar conditions.

Diagnostic Tests

While specific tests for this particular type of tumor may not be widely available or documented, the following tests may be relevant for diagnosing similar conditions:

  • Blood tests: Elevated somatostatin hormone levels in blood tests may indicate the presence of a somatostatin-producing tumor (1).
  • Imaging tests: Computed Tomography (CT), Magnetic Resonance Imaging (MRI), or Somatostatin Receptor Scintigraphy (SRS) may be used to detect tumors and confirm diagnosis (6, 8).
  • Chromogranin A test: This test analyzes the levels of Chromogranin A and other hormones in the blood, which may indicate the presence of a neuroendocrine tumor (4).

Rare Tumors

It's worth noting that small intestinal delta cell somatostatin-producing tumors are rare, with an estimated incidence of 1 in 40 million individuals per year (12). As such, diagnostic tests and procedures for this specific condition may not be widely available or well-documented.

References

  • [1] Elevated somatostatin hormone levels in blood tests may indicate the presence of a somatostatin-producing tumor.
  • [4] Chromogranin A test analyzes the levels of Chromogranin A and other hormones in the blood, which may indicate the presence of a neuroendocrine tumor.
  • [6] Computed Tomography (CT), Magnetic Resonance Imaging (MRI), or Somatostatin Receptor Scintigraphy (SRS) may be used to detect tumors and confirm diagnosis.
  • [8] The diagnosis is made by a fasting serum somatostatin level greater than 100 pg/mL, with CT scan, MRI, and endoscopic ultrasound often helping in the diagnostic process.

Treatment

Differential Diagnosis

Differential Diagnosis of Somatostatinoma

A differential diagnosis for an obsolete small intestinal delta cell somatostatin-producing tumor involves considering various conditions that may present with similar symptoms and characteristics. Based on the search results, here are some possible differentials:

  • Other neuroendocrine tumors (NETs): NETs can arise from various parts of the body, including the pancreas, duodenum, and small intestine. These tumors can produce a range of hormones, including somatostatin, and may present with similar symptoms to somatostatinoma [1, 8].
  • Pheochromocytomas and paragangliomas: These rare tumors can also produce somatostatin, but hormone excess is not typically associated with somatostatinoma syndrome [13].
  • Other small intestinal tumors: Tumors such as carcinoids, gastrinomas, and VIPomas may present with similar symptoms to somatostatinoma, including abdominal pain, weight loss, and diarrhea [5, 7].

Key Features to Consider

When considering a differential diagnosis for an obsolete small intestinal delta cell somatostatin-producing tumor, the following key features should be taken into account:

  • Location: The tumor is located in the small intestine, which may help differentiate it from other NETs that arise from the pancreas or duodenum.
  • Histology: The tumor is composed of delta cells, which produce somatostatin. This histological feature can help distinguish it from other types of NETs.
  • Symptoms: The patient presents with symptoms such as abdominal pain, weight loss, and diarrhea, which are similar to those seen in somatostatinoma.

References

[1] Somatostatinoma is a rare neuroendocrine tumor (NET) with an incidence of 1 in 40 million persons [9]. [8] Somatostatin expressing D cell NETs: tubuloglandular pattern; may have psammoma bodies [15]. [13] Pheochromocytomas and paragangliomas also sometimes produce and secrete somatostatin, but hormone excess is not typically associated with somatostatinoma syndrome [13].

Additional Information

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