A rare case of poorly differentiated mixed neuroendocrine-nonneuroendocrine tumor of the caecum with long term survival: A case report

Abstract

A 59-year-old woman presented with flushing attacks accompanied by tachycardia and hypotension, which lasted approximately 30 to 60 minutes, underwent 18 years ago a gastrointestinal tumor resection. The histologic examination revealed a poorly differentiated mixed neuroendocrine/adenocarcinoma located in the caecum with regional metastases. Postoperatively, the patient received combined chemotherapy of 5-fluorouracil with interferon for six months and since has remained asymptomatic. Her examination revealed positivity for chromogranin A (CgA) and a-Fetoprotein (aFP) (580 ng/24 h, normal range 27–94, and 10 IU/mL, normal range 0–6, respectively). Urinary 5-hydroxy indole acetic acid excretion was remarkably high (41.8 mg/24 h, normal range 2–10 mg/24 h). An abdominal Magnetic Resonance Imaging scan revealed multiple focal loci in the liver whose histological examination revealed a carcinoid tumor confirmed by an Octreoscan. Additional uptake was noted on the right shoulder and the right sternum-clavicle joint confirmed by Tc-99m MDP scan. The patient received somatostatin analogue therapy followed by long-acting release octreotide analogue therapy (30 mg/month) showing a partial improvement of relevant biomarkers. Two years later, carcinoid syndrome symptoms reappeared and due to the tumors expression of somatostatin receptors the patient received peptide receptor radionuclide therapy with 177Lu-DOTATATE that resulted in both clinical and biochemical improvements.

Sternum Metastases: From Case-Identifying Strategy to Multidisciplinary Management

“…In spite of this, surgery remains the first choice, if feasible (even debulking procedures might help). In one case of a 59-year-old female with carcinoid syndrome related to a caecum primary tumour, the patient was treated for 6 months with chemotherapy and interferon, followed by Octreoscan-based confirmation of liver and bone metastases (including at the level of the right sternum–clavicle joint); thus, a switch to octreotide LAR was made, allowing for stabilisation of the disease for 2 years when the disease progression required PRRT (177Lu-DOTATATE) [ 68 ].…”

“…Overall, we identified 14 studies (of more than 1 subject/article) [ 18 , 20 , 21 , 22 , 24 , 51 , 53 , 64 , 66 , 70 , 72 , 73 , 74 , 75 ] and 34 single case reports [ 14 , 15 , 16 , 17 , 19 , 23 , 25 , 26 , 38 , 41 , 42 , 43 , 47 , 52 , 55 , 56 , 59 , 60 , 62 , 68 , 71 , 76 , 77 , 78 , 89 , 98 , 99 , 100 , 101 , 102 ] within our methods with respect to sternal metastases (n = 48 papers) ( Table 5 ).…”

“… Overview of published case reports (N = 34) according to our methods [ 14 , 15 , 16 , 17 , 19 , 23 , 25 , 26 , 30 , 32 , 33 , 37 , 38 , 41 , 42 , 43 , 47 , 52 , 55 , 56 , 59 , 60 , 62 , 68 , 71 , 76 , 77 , 78 , 89 , 98 , 99 , 100 , 101 , 102 ]. Abbreviations: N = number of patients; MiNEN = mixed neuroendocrine–non-neuroendocrine tumour.…”

“…Table 5. Single case reports of sternal metastases according to our methods; the articles are cited starting with the most recent time of publication [14][15][16][17]19,23,25,26,30,32,33,37,38,[41][42][43]47,52,55,56,59,60,62,68,71,[76][77][78]89,[98][99][100][101][102]. The case we mentioned brought up the importance of addressing a sternal biopsy into the larger frame of multi-lined, complex management.…”

“…The originating tumour profile revealed that the most frequent types were mammary cancer (N = 8, all females) and thyroid cancer (N = 9, both women and men), followed by bladder carcinoma (N = 3), lung carcinoma (N = 2), and malignancy of renal origin (N = 2). One case was identified for each of the following sites: adenoid cystic carcinoma of the jaw, malignant melanoma, caecum MiNEN, brain and an extracranial meningioma, tongue carcinoma, cholangiocarcinoma, osteosarcoma, and hepatocellular carcinoma [14][15][16][17]19,23,25,26,30,32,33,37,38,[41][42][43]47,52,55,56,59,60,62,68,71,[76][77][78]89,[98][99][100][101][102] (Figure 9). [14][15][16][17]19,23,25,26,30,32,33,…”