MOFFITT ONCOLOGY REPORT PEARLS 8/5/16: Neuroendocrine Tumors and HLH!

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):
    • Carcinoid
    • Well-differentiated pancreatic islet cell tumor (PNET)
      • Gastrinoma
      • Insulinoma
      • Glucagonoma
      • Somatostatinoma
      • VIPoma
    • Medullary thyroid cancer
    • Paraganglioma
    • Pheochromocytoma

 

  • Aggressive neuroendocrine carcinomas (NECs = poorly differentiated):
    • Small/large cell carcinoma (lung and many other primary sites)
    • Merkel cell tumor of the skin
    • Neuroblastoma

 

**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

 

HLH Review:

Associated with infections, malignancy, rheumatologic disorders, and immunodeficiencies.

Need 5 out of 8 of the following:

  1. Fever > 38.5
  2. Splenomegaly
  3. Cytopenias (2 out of 3 of hgb <9, plt <100, ANC <1000)
  4. Triglycerides >265 or fibrinogen <150
  5. Ferritin >500 (>3000 more indicative)
  6. Low NK cell activity
  7. Elevated soluble CD25 (soluble IL-2 receptor)
  8. 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.

 

Evernote: https://www.evernote.com/shard/s272/sh/942c1390-e9f1-4499-b17d-79c539cfb582/2603e859b85a57f1b39384948ec94512

Advertisements

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s