Dear Dr.: Thank you for the online referral and the copy of the medical records for this patient through Medical Opinion services. I had the opportunity to review the patient’s medical records.
To summarize, the patient is a 33 year old woman that was discovered to have a 2cm pancreatic lesion. One month later a CT scan showed a 1.5cm lesion between the pancreas and the renal vein and a 2cm lymph node near the pancreas. There was evidence of thickening of the stomach plicae. CT of the brain revealed a small hypointense lesion between the anterior and posterior hypophysis. An ultrasound of the neck revealed hyperplastic parathyroid glands.
The patient underwent removal of the pancreatic lesion and lymph node dissection. The pathology report revealed an insulinoma in the pancreas with lymph node metastasis and a gastrinoma of the pancreas. The patient underwent surgery again to remove the distal pancreas and spleen. The pathology report revealed multiple benign endocrine tumors. The patient underwent a parathyroidectomy which revealed multiple adenomas.
Octreoscan revealed uptake in the epigastric area. Two months later, CT of the abdomen revealed a 1.1cm lesion in the pancreas. Octreoscan was repeated and revealed a pancreatic lesion and suspected repetitive abdominal lesions. A parathyroid scintigraphy revealed hyperfunctioning parathyroid tissue. A repeat Octreoscan revealed recurrence in the pancreas, epigastric region, left paracolic and paraumbilical regions as well as the parathyroid glands. The patient is currently asymptomatic.
It appears that the patient has multiple endocrine neoplasia Type I (MEN-I). She has parathyroid adenomas, pancreatic insulinoma/ gastrinoma, and suggestion of pituitary adenoma on CT of the brain. The family history is not given which would be of interest since this is an autosomal dominant disease; however, if there is no family history, the patient may represent the index case of this disease.
Hyperparathyroidism is the most common manifestation of MEN-I. We do not have information on calcium levels and parathyroidism levels; but, the patient is currently asymptomatic. There is a high rate of recurrent hyperparathyroidism after apparently successful subtotal parathyroidectomy in patients with MEN-I and it appears from imaging studies that the patient may have recurrent hyperparathyroidism at this time.
Pituitary adenomas develop in approximately 15 -20% of patients with MEN-I screened with CT or MRI. The most common type of adenoma in MEN-I is a prolactinoma. Since the patient is currently asymptomatic, with no signs of enlarged adenoma, there is no clinical indication for intervention with respect to the suspected pituitary adenoma at this time.
There is an effective treatment for hyperparathyroidism and pituitary disease in MEN-1; the life-threatening manifestation of MEN-1 is the malignancy of pancreatic islet cell tumors. Pancreatic islet cell tumors in MEN-1 often synthesize multiple hormones as seen with this patient. Patients do not always have clinical symptoms due to the fact that many of these tumors may be defective in their hormone processing apparatus or have an inefficient secretory mechanism.
MEN-I-related gastrinoma tumors are multifocal, often small and easily overlooked, making surgical removal largely unsuccessful. The risk of death from malignant spread of MEN-1-associated gastrinoma appears to be less than that for isolated gastrinoma. Local lymph node metastases are common. Insulinomas in MEN-1 are also small, multiple, and associated with the simultaneous presence of other islet cell tumors.
Although the patient is asymptomatic at this time, unfortunately, a recent Octreoscan indicates evidence of recurrent disease in the pancreas and metastatic disease in abdominal lymph nodes. There is also evidence of hyperfunctioning parathyroid tissue.
With respect to medical treatment, I recommend checking calcium and parathyroid hormone levels to determine the extent of hyperparathyroidism. Surgical intervention for hyperparathyroidism is not warranted again at this time. Medications such as estrogen plus progestin, bisphosphonates, and raloxifene inhibit bone resorption and can increase bone density and lower serum calcium concentrations in patients with hyperparathyroidism. Other medications, such as calcimimetics or vitamin D analogues suppress parathyroid hormone release, or counteract hyperparathyroidism effects at the level of PTH receptors. With respect to the metastatic islet cell tumor, I recommend testing baseline gastrin and insulin. It appears that the patient´s disease has been progressing over the past year. Because of her disease progression, I recommend treating with Octreotide LAR (sandostatin) monthly and increasing the dose as tolerated. Gastrin and insulin levels should be checked at 3 month intervals to determine response to treatment. If the patient’s disease progresses while on Octreotide LAR, a chemotherapy combination of platinum and etoposide would be recommended. Enrollment in a clinical trial, if available, would also be appropriate.
It is difficult to make estimates regarding prognosis as this largely depends upon her response to therapy. The patient may have a very favorable response to Octreotide and her disease may be manageable for quite some time.
Thank you for allowing me to participate in the online care of your patient. I wish you and the patient the best. I would be happy to see this patient in my practice. If we can be of any further help to you, please contact me through Medical Opinion.