Rare-Site Primary Soft-Tissue Neuroendocrine Tumor with Metastases and Near-Complete Resolution with 177Lu-DOTATATE: Documenting a Promising Clinical Application of Peptide Receptor Radionuclide Therapy.

Neuroendocrine tumors (NETs) of the skin or soft tissue are rare (mostly described as sites of metastasis), whereas primary soft-tissue NETs are extremely rare; they are usually diagnosed at advanced stages with distant metastases because of their indolent nature. We herein describe our experience with 2 such cases. In the first case, the NET originated in the retroperitoneal soft tissue, and in the second case, the patient was a middle-aged lady with NET arising from soft tissue in the pelvis. Both patients were treated with 177Lu-DOTATATE in view of their somatostatin receptor-expressing metastatic lesions, demonstrating an excellent outcome as reflected by a complete metabolic response and near-complete anatomic response to the administered peptide receptor radionuclide therapy. The noteworthy factors of the reported cases were, first, unusual sites of primary tumor and, second, near-complete to complete symptomatic, anatomic, and metabolic resolution of the recurrent primary tumor and metastatic lesions with peptide receptor radionuclide therapy alone. NETs arising from rare anatomic locations are usually nonfunctioning, with good clinical outcomes, and 177Lu-DOTATATE peptide receptor radionuclide therapy can be considered promising in patients with metastatic or advanced disease.