Medical Diagnosis: Multiple endocrine neoplasia Type I (MEN-I) of the pancreas.
A 33 year old female was diagnosed with endocrine carcinoma of the pancreas. No information regarding medical history was reported by the patient.
A CAT scan of the pancreas demonstrated a hyperdense oval lesion with a central hypodense area having a maximum diameter of about 2cm. This was at first was compatible with an endocrine type primitive productive process.
Based on these results, a further series of diagnostic tests were carried out (CAT scan of brain, ultrasound of neck and upper abdomen with contrast medium, an ultrasound of thyroid/parathyroid, full abdomen and upper abdomen, CAT scan of abdomen). These tests showed various pathological abnormalities listed below.
These abnormalities can be summarized as follows:
- A pancreatic formation of approximately 2cm, with a hypointense NMR signal, which with contrast medium showed an increased intensity of the signal in the arterial phase and a slight central dishomogeneity.
- An oval formation of approximately 1.5cm located outside the pancreas between the head of the pancreas and the left renal vein, in proximity to the outlet to the vena cava. Both of these lesions were interpreted as small hypervascularized expansive formations.
- An increase in thickness of stomach walls with accentuated plicae.
- A lymph node of about 2cm, in proximity with the head of the pancreas.
- In the brain, a hypo-intense oval area of approximately 4-5mm located at the intersection between the anterior and posterior hypophysis, suspected to be a hypophysis microadenoma.
- In the neck, 3 hyperplastic parathyroids were identified caudally to the thyroid on the right, and on the left 2 hyperplastic parathyroids were shown near the esophagus immediately behind the laryngeal nerve.
- Right renal cyst.
A surgical procedure was carried out to remove the growth in the pancreas by intra-aortocaval retropancreatic lymphadenectomy.
Histology tests on the surgical samples listed below showed the following:
- No neoplasia in intra-aortocaval retroduodenal lymph nodes.
- Nodule on the large omentum: No neoplasia in lymph node.
- No neoplasia in lymph node at inferior margin of tail of pancreas.
- Neoplasia at head of pancreas, suspected insulinoma (oval nodule of 3 x 2.5cm). Clearly differentiated lymph node metastasis of endocrine carcinoma.
- No neoplasia in lymph node along left gastric artery.
- Neoplasia of tail of pancreas, suspected gastrinoma. Clearly differentiated endocrine carcinoma.
Another surgical procedure of distal pancreatectomy was carried out with removal of tail and part of body of pancreas and total splenectomy. The histology test showed the following:
No neoplasia in Tripod lymph node. No neoplasia in hepatic peduncle lymph nodes. Surgical sample (spleen and pancreas): Clearly differentiated multiple endocrine benign tumors.
A further surgical procedure of parathyroidectomy was carried out and the results of the histology test were as follows:
Right inferior parathyroid adenoma: Thyroid macrofollicular adenoma.
Parathyroid adenoma.Lymph node with sinus histiocytosis.
Whole body scintigraphy showed an irregular image of trace hyper-accumulation in the right paramedian anterior epigastric area, suggesting a lesion expressing somatostatin receptors in apparent slight progression.
The complete abdominal CAT scan showed a partly calcified hyperdense formation in the pancreas, near the mesenteric vein, having an estimated dimension of 11 mm, indicative of base pathology localization. These results were subsequently confirmed by an ultrasound.
The results of the whole body scintigraphy were as follows: Results were substantially the same as the previous follow-up and were compatible with the presence of a pancreatic lesion expressing somatostatin receptors and suspected repetitive abdominal lesions.
The results of the parathyroid scintigraphy were as follows: Results were compatible with the presence of hyper-functioning parathyroid tissue.
The results of the whole body scintigraphy with immune and receptor traces confirmed the presence of a suspected recurrence of neuroendocrine heteroplasia in the pancreas. The results also showed areas expressing somatostatin receptors in the abdomen (epigastric region with localization at the head of the pancreas and in the left paracolic and homolateral paraumbilical regions) and parathyroid.
The patient reports that she is currently completely asymptomatic.