Malignant transformation of glucagonoma with SPECT/CT In-111 OctreoScan features

Abstract

Rationale:Glucagonoma is an uncommon disease but it has been associated with a pattern of symptoms defined as glucagonoma syndrome. These symptoms, if promptly recognized, could help to speed up the diagnosing process.Patient concerns:We report a case of a 68-year-old woman with a pancreatic glucagonoma. Her symptoms at the onset were typical of the glucagonoma syndrome.Diagnoses:After a significant weight loss, she underwent a computer tomography scan of the abdomen, which showed a hypervascular lesion of the tail of the pancreas and hypervascular lesions of the liver. An ultrasound guided biopsy was performed and pathology was consistent with glucagonoma. Her blood glucagon levels were elevated.Outcomes:She was treated with chemotherapy and somatostatin analogs. After 4 years, the disease had a malignant transformation, and metastases suddenly started to grow up. She stopped being responsive to treatment and eventually passed away.Lessons:Due to its rarity, clinical diagnosis is challenging and generally it comes after a long interval since the onset of symptoms. Awareness of physicians and dermatologists of the characteristic necrolytic migratory erythema, and of the other symptoms, often leads to early diagnosis.

Emerging therapies for advanced insulinomas and glucagonomas

“…Glucagonomas can be very aggressive such as in the case of a 68-year-old woman who received six cycles of dacarbazine followed by SSA, but when relapsed 4 years later, none of the interventions such as TAE, 5-FU, and platinum-based treatment could control her disease (Corrias et al 2017). Moreover, there are cases resistant to treatment such as a 57-year-old man who achieved SD after a sequence of treatment with different chemotherapeutical regimens including cisplatin, etoposide, 5-FU, irinotecan, and TEMCAP together with octreotide.…”

Pancreatic Neuroendocrine Tumors: A Literature Review

“…According to the classification of tumors of the digestive system of the World Health Organization (WHO), Glucagonomas are functioning PNETs, and manifest with typical multiple symptoms, such as weight loss of 10 to 15kg, diarrhea, diabetes mellitus, venous thromboembolism, migratory necrolytic erythema, rash, steatorrhea, anemia, depression, and neuropsychiatric factors. However, early diagnosis, which could provide curative resection, remains difficult due to the uncommonness of this disease [22,23,25]. Glucagonomas similarly affect men and women and are usually large tumors (>4cm), whose anatomical site is frequent in the distal part of the organ (body and pancreatic tail).…”

“…Glucagonomas similarly affect men and women and are usually large tumors (>4cm), whose anatomical site is frequent in the distal part of the organ (body and pancreatic tail). They are commonly diagnosed from ages 40 to 50 years, and 50% of patients present metastases at the time of diagnosis [22,23,26].…”

“…Another useful of scintigraphy is to evaluate the functional status of the tumor and the secondary location, providing appropriate therapy with peptide receptor radionuclide (PRRT). Treatment may vary depending on the stage of the disease, but currently, surgical resection is the only one that presents a cure chance for glucagonoma (NME can disappear within 1 week after surgery) [22,25]. Somatostatin analogs and amino acid solution infusion result just in symptom relief.…”

“…Patients may feel significant improvement after treatment and cognitive improvement after surgery, suggesting that neuropsychiatric symptoms are due to the high level of glucagon [25,26]. Despite the malignancy of the disease, overall survival even in patients with metastasis ranges from 3 to 8 years [22]. A patient with glucagonoma may have Necrotic Migratory Erythema (NME), characteristic of excess glucagon serum.…”