Thanks to Vaibhav for presenting a super interesting (and sad) case, and Pat Cornett for guiding us through heme/onc report this morning. We talked about neuroendocrine neoplasms and HLH, or the “bread and butter” of Moffitt as I call them. Pearls below!
Neuroendocrine neoplasms: Histology is much more important than primary site (want tissue)!
- Indolent neuroendocrine tumors (NETs = well differentiated):
- Well-differentiated pancreatic islet cell tumor (PNET)
- Medullary thyroid cancer
- Aggressive neuroendocrine carcinomas (NECs = poorly differentiated):
- Small/large cell carcinoma (lung and many other primary sites)
- Merkel cell tumor of the skin
**Pearl: Most NETs of unknown primary present with liver metastases.
NET workup and treatment:
- CT abdomen, OctreoScan, tumor markers (chromogranin A for all NETs, specific hormone levels for functioning tumors)
- EGD (EUS) and colonoscopy if primary site still not identified
- Tx: Octreotide for carcinoid or other hormone-secreting tumors, Everolimus
NEC workup and treatment:
- PET/CT chest/abdomen/pelvis, MRI brain
- Bronchoscopy if primary site still not identified, other testing guided by symptoms
- Tx: Carboplatin/cisplatin + etoposide
Associated with infections, malignancy, rheumatologic disorders, and immunodeficiencies.
Need 5 out of 8 of the following:
- Fever > 38.5
- Cytopenias (2 out of 3 of hgb <9, plt <100, ANC <1000)
- Triglycerides >265 or fibrinogen <150
- Ferritin >500 (>3000 more indicative)
- Low NK cell activity
- Elevated soluble CD25 (soluble IL-2 receptor)
- Hemophagocytosis on biopsy (BM, spleen, LN, liver)
Untreated survival on order of months (progressive multiorgan failure).
50% survival rate with treatment.
Tx: Etoposide and dexamethasone, hematopoietic stem cell transplant if refractory.