Cutaneous metastasis as an initial presentation of a non-functioning pancreatic neuroendocrine tumor

Abstract

Non-functioning pancreatic neuroendocrine tumors (NF-PNETs) are rare tumors that account for 2% of all pancreatic malignancy. About 60% of NF-PNETs present distant metastases and usually hepatic metastases. However, cutaneous metastases are very rare. Herein, we report our experience with a 60-year-old male who visited our outpatient clinic with a mass on his left hip. An abdominal computerized tomography scan demonstrated not only a left hip mass and an enlarged left inguinal lymph node, but also a huge heterogeneous enhancing mass on the pancreas. Initially, we removed the metastatic lesions, which was a small cell neuroendocrine carcinoma with 50% of the Ki-67 index in the histopathological report. After 3 wk, we performed a total pancreatectomy and a total gastrectomy. Four weeks after the 1(st) operation, we detected a recurrence at the operative bed on his left hip, and subsequently removed the recurring mass. The patient was receiving chemotherapy based on etoposide and cisplatin treatment.

Unexpected response to palliative radiotherapy for subcutaneous metastases of an advanced small cell pancreatic neuroendocrine carcinoma: a case report of two different radiation schedules

“…Cases of skin (cutaneous/subcutaneous) metastases derived from neuroendocrine tumors of the lung [3][4][5][6][7][8], gastrointestinal [2,[9][10][11] and genitourinary tract [12][13][14][15] as well as of other origins or with unknown primary tumor site [16][17][18] have been exceptionally reported. In line with observations and revisions by Amorim et Al.…”

Rare breast and subcutaneous metastases from pancreatic neuroendocrine tumor: a case report

“…PNETs derived from different neuroendocrine cells are a clinically rare and heterogeneous disease of the pancreas, accounting for 7% of all neuroendocrine tumors. The annual incidence of PNETs in the USA is estimated to range between 2 and 5 cases per 1,000,000 individuals, but, as stated above, the number of diagnosed cases has been increasing in recent years [2, 3]. Risk factors for PNETs include smoking, a high body mass index, and a positive family history which accounts for a variable percentage of patients with inherited syndromes, such as multiple endocrine neoplasia type 1 (known as MEN1) [9].…”

“…The development of molecular targeted agents has changed the landscape of treatment for PNETs [17]. According to the European Neuroendocrine Tumor Society (commonly known as ENETS) consensus guidelines, resection of metastases of grade 3 pancreatic neuroendocrine carcinoma is generally not recommended but may be considered in individual cases with isolated resectable metastases [2]. Active surveillance, especially for non-functioning tumors < 2 cm in size, should be considered although surgery remains the mainstay of treatment [9].…”

“…Neuroendocrine tumors represent a group of rare neoplasms, with an overall incidence of approximately 5.25/100000 [1]. The pancreatic neuroendocrine tumors (PNETs) represent only 1–2% of all pancreatic malignancy tumors, with incidence of 1–5 cases per million, mainly afflicting adults between the ages of 30 to 60 years; however, recent advances in imaging technologies and application of endoscopic ultrasound have led to an increase in the number of diagnosed PNETs [2–5]. Case reports in the literature have indicated that the most common sites of these tumors are the gastrointestinal tract, lung, adrenal gland, and thyroid gland [1, 6].…”

Cutaneous Manifestations in Pancreatic Diseases—A Review

“…Skin metastasis has been mostly reported in adenocarcinoma of the pancreas, but other histological subtypes have also been associated with cutaneous spread–adenosquamous cell carcinoma, mucinous cystadenocarcinoma, neuroendocrine carcinoma or VIP (vasoactive intestinal polypeptide) tumor [ 7 , 29 , 30 ].…”