Paraganglioma and Pheochromocytoma

Paraganglioma and Pheochromocytoma click to access explanations in the original CAP template

Useful information to be included from the case notes:
1. Clinical history
2. Functional status
--- Biochemically functioning
----- Metanephrine and / or adrenaline
----- Normetanephrine and / or noradrenaline
----- 3-Methoxytyramine and / or dopamine
--- Nonfunctional (biochemically non-functioning)
3. Tumor Scintigraphy or PET Avidity
- DOTATATE or DOTATOC PET
- 123I-metaiodobenzylguanidine (MIBG) scintigraphy
4. Anatomical location from imaging
5. Tumor sizes from imaging

- Procedure:
- Tumor integrity:
- Tumor Number:
- Tumor Location:
- Tumor size:

Microscopic descriptions:

- Tumor histology:

- Histologic type:
- Tumor Proliferative Activity:
	- Ki-67 Labeling Index: Selecting multiple small hot spots (at least 500 tumor cells) from different hot spot regions of the tumor rather than a single larger area of the same tumor are recommended.
	+ Mitotic Count (per 2 mm²): Using 22 mm eyepiece at 40x objective, count active proliferative areas for 42 fields (equals 10 mm²), divide by 5 to get mitotic count for 2 mm²
- Tumor Necrosis:
- Angioinvasion (vascular invasion):
- Lymphatic invasion:
+ Perineural invasion:
- Tumor Extent:	Not applicable Tumor confined to adrenal with no adrenal capsule invasion or no peritumoral soft tissue invasion Adrenal capsule invasion without extra-adrenal tumor spread Peri-adrenal or peri-tumoral soft tissue invasion Adjacent organ / tissue invasion (e.g., liver, pancreas, spleen, kidney) (specify): Other (specify): Cannot be determined:
+ Immunohistochemical studies
	+ Keratins
	+ Chromogranin-A
	+ Tyrosine Hydroxylase
	+ GATA3
	+ S100 and / or SOX10-positive Sustentacular Cell Network
	+ SDHB
	+ Alpha-inhibin
	+ CAIX
	+ FH
	+ 2-SC
	+ ATRX

Surgical margins of excision specimen:

- Surgical margins are all clear:
- Surgical margins are involved:

Regional lymph nodes:

- Number of regional lymph nodes examined:
- Number of lymph nodes involved (specify locations):
+ Extranodal extension:
+ Size of largest metastatic deposit:
+ Size of largest lymph node involved:

Distant Metastasis: Not applicable Not identified Bone Lung Liver Other (specify): Cannot be determined:

Pathologic Stage Classification (AJCC 8e):

Applied to all sympathetic paragangliomas and pheochromocytomas. Currently there is no defined TNM Classification for parasympathetic paragangliomas and composite paragangliomas / pheochromocytomas but users can apply the TNM staging below (based on the tumor size cutoff of 5 cm and the status of invasion) to facilitate uniform data collection.

- TNM Descriptors:	m (multiple primary synchronous tumors in a single organ) r (recurrence) y (post-neoadjuvant therapy)
- Primary tumor (pT):
- Regional lymph nodes (pN):
- Distant metastasis (pM):

Additional pathologic findings:

- Adrenal Medullary Hyperplasia (required for all adrenalectomy specimens):

None identified Other tumors (specify): Other (specify):

- Additional notes: