Approach to the patient with aldosterone-producing adrenocortical carcinoma / 中华内分泌代谢杂志

Aldosterone-producing adrenocortical carcinoma (ACC) is a rare endocrine malignancy. Only a few cases are reported in China. This systematic review investigated the diagnosis and treatment strategy of aldosterone-producing ACC through a recent case of the disease. A case of a 49-year-old female who diagnosed with aldosterone-producing ACC by hormonal assays, medical imaging and pathology. Her condition has been alleviated after surgery. Aldosterone-producing ACC is a rare malignancy with limited treatment options and surgery is the primary treatment strategy.

Surgical treatment of pancreatic endocrine tumors in multiple endocrine neoplasia type 1

Surgical approaches to pancreatic endocrine tumors associated with multiple endocrine neoplasia type 1 may differ greatly from those applied to sporadic pancreatic endocrine tumors. Presurgical diagnosis of multiple endocrine neoplasia type 1 is therefore crucial to plan a proper intervention. Of note, hyperparathyroidism/multiple endocrine neoplasia type 1 should be surgically treated before pancreatic endocrine tumors/multiple endocrine neoplasia type 1 resection, apart from insulinoma. Non-functioning pancreatic endocrine tumors/multiple endocrine neoplasia type 1 >1 cm have a high risk of malignancy and should be treated by a pancreatic resection associated with lymphadenectomy. The vast majority of patients with gastrinoma/multiple endocrine neoplasia type 1 present with tumor lesions at the duodenum, so the surgery of choice is subtotal or total pancreatoduodenectomy followed by regional lymphadenectomy. The usual surgical treatment for insulinoma/multiple endocrine neoplasia type 1 is distal pancreatectomy up to the mesenteric vein with or without spleen preservation, associated with enucleation of tumor lesions in the pancreatic head. Surgical procedures for glucagonomas, somatostatinomas, and vipomas/ multiple endocrine neoplasia type 1 are similar to those applied to sporadic pancreatic endocrine tumors. Some of these surgical strategies for pancreatic endocrine tumors/multiple endocrine neoplasia type 1 still remain controversial as to their proper extension and timing. Furthermore, surgical resection of single hepatic metastasis secondary to pancreatic endocrine tumors/multiple endocrine neoplasia type 1 may be curative and even in multiple liver metastases surgical resection is possible. Hepatic trans-arterial chemo-embolization is usually associated with surgical resection. Liver transplantation may be needed for select cases. Finally, pre-surgical clinical and genetic diagnosis of multiple endocrine neoplasia type 1 syndrome and localization of multiple endocrine neoplasia type 1related tumors are crucial for determining the best surgical strategies in each individual case with pancreatic endocrine tumors.

Clinical profile of insulinoma: analysis from a tertiary care referral center in India.

Objective To describe the clinical presentation, localization techniques, surgical procedures and outcome in patients with insulinoma. Methods Retrospective analysis of case records of patients diagnosed with insulinoma between January 1993 and June 2009 at a tertiary-care hospital was done. Seventeen patients underwent diagnostic 72-h fast. The sensitivity of computed tomography (CT) of pancreas was judged using intraoperative findings as the gold standard. Results Twenty-six patients (19 women) with mean age 42.2 years (median age 39.5 years) with biochemical/ imaging evidence of insulinoma were included. All patients who underwent 72-h fast developed symptomatic hypoglycemia within 48 h. The sensitivity and positive predictive value (PPV) of CT of pancreas was 68.4% and 92.8%, respectively. Twenty patients underwent surgery, of whom four were operated on without preoperative localization. Four patients had recurrence of symptoms. Of the six patients who were not operated upon, the lesion was localized in four. Conclusion This audit shows that all patients with insulinoma develop hypoglycemia within 48 h of fasting. CT of pancreas localizes the lesion in two thirds of cases and would be a useful initial investigation in patients with suspected insulinoma.

Diagnosis and surgical management of functional pancreatic endocrine tumors: a report of 45 cases / 中华消化外科杂志

Objective To investigate the diagnosis and treatment of functional pancreatic endocrine tumors (PETs). Methods The clinical data of 45 patients with functional PETs who were admitted to the Southwest Hospital from January 1998 to December 2008 were retrospectively analyzed. Etiologic and localization diagnosis were made preoperatively according to the manifestation and the results of color doppler ultrasound and computed tomography, respectively. Eight patients received resection of the body and tail of the pancreas and spleen, 32 received tumor enucleation, one received resection of the pancreaticobiliary junction and four received pancreaticoduodenectomy. All patients received chemotherapy after the operation. Results Thirty-four cases of the PETs were benign and the rest eleven cases were malignant. Eight cases of the PETs were in the head of the pancreas,26 in the tail of the pancreas, seven in the body of the pancreas and four cases were with multiple PETs. The diameters of the PETs were 0. 3-5.0 cm, and the diameters of the PETs in 19 cases were above 2.0 cm. Eight patients were complicated with pancreatic leakage, two with incision infection and one with abdominal infection. Of the 33 patients with insulinoma, the blood glucose of five patients with multiple PETs was still abnormal after the operation, three patients underwent reoperation and the other two were treated by diazoxide to control the blood glucose in the normal range. The clinical symptoms of the seven patients with gastrinoma disappeared after the operation, and the gastric ulcer was healed, the 12-hour gastric juice volume and the level of the gastric acid were in the normal range after a continuous treatment with proton pump inhibition agents for 6 months. Necrotizing or migratory rash and diabetes of the four patients with glucagonoma were cured three weeks later, and the level of the amino acid was back to normal. Diarrhea and electronic disturbance of one patient with vasoactive intestinal peptide tumor were alleviated after the operation. Thirty-nine patients were followed up for 20-120 months. Of the 32 patients with benign PETs, two patients had tumor recurrence, and three patients died of other diseases. Of the seven patients with malignant PETs, two patients survived, and three patients died of hepatic metastasis or tumor recurrence, two patients died of other diseases. Conclusions Surgical treatment is effective for the treatment of functional PETs. Palliative resection of the tumor also can obviously improve the life quality of patients with fuctional PETs.

Tumores endócrinos do ciclo gravídico puerperal: [revisão] / Endocrine tumors of the puerperal pregnancy cycle: [review]

Os tumores endócrinos têm maior incidência nas mulheres em idade fértil, mas mesmo nestas pacientes sua frequência é pequena e, em geral, são clinicamente silenciosos, só manifestando-se no decorrer da gestação. Neste artigo de revisão, os autores apresentam os principais tumores do período gestacional, como os tumores hipofisários, o feocromocitoma, os tumores de tireoide e o tumor do ovário e relatam ainda suas características clínicas e formas de tratamento.

Neoplasia endocrina múltiple tipo 1 / Multiple endocrine neoplasia type 1

Multiple endocrine neoplasia type 1 (NEM1) is an uncommon autosomal dominant disease caused by an alteration of menin, a tumor suppression protein and is characterized by the presence of primary tumors in at least two different endocrine tissues. It is described as the “three P disease”since it involves mainly the pituitary, parathyroid and pancreas. However more than 20 different tumor locations have been described. Most tumors are benign and primary hyperparathyroidism is the first manifestation of the disease in 90 percent of cases enteropancreatic tumors appear in approximately 60 percent of patients and pituitary adenomas, usually prolactinomas, in 30 percent. Skin lesions, non functional adrenal adenomas and neuroendocrine tumors such as carcinoid are also part of the disease. We describe the pathogenesis, clinical presentation, diagnosis and treatment of NEM1.

Localização de insulinoma pela injeção seletiva intra-arterial de cálcio / Localization of insulinoma by selective intraarterial calcium injection

Insulinomas are rare endocrine tumors with an estimated incidence of 1(one) per million. Optimal therapy for all islet tumors of the pancreas is curative surgical resection. However, previous reports have show that, in the absence of preoparative localization, insulinoma may not be found intraoperatively in about 20 percent of patients. With current imaging technology, including serial computed tomography (CT), magnetic resonance imaging (MRI) and ultrasonography, localization of insulinomas less than 2cm remains inadequate. This case report shows that selective intraarterial calcium injection with hepatic venous sampling for insulin levels measured is a efficient technique for the localization of insulinomas.