Local-regional recurrence of sporadic or syndromic abdominal extra-adrenal paraganglioma: incidence, characteristics, and outcome.
BACKGROUND
Operative excision of abdominal extra-adrenal paragangliomas (EAPs) does not preclude the late development of local-regional recurrence. We describe the incidence, characteristics, and outcome of this rarely reported feature.


METHODS
Retrospective analysis of local-regional recurrence that occurred during follow-up of 51 consecutive patients operated for a sporadic (n = 26) or hereditary (n = 25) EAP.


RESULTS
Seven patients with a sporadic or syndromic EAP (n = 4: von Hippel-Lindau syndrome and SDHB, SDHC, and SDHD gene mutations) underwent reoperation for a local-regional recurrence after a median time of 46 months (interquartile range [IQR], 16-100). The Kaplan-Meier estimated incidence of local-regional recurrence (+/- standard error of the mean) reached 15% +/- 7% at 5 years and 23% +/- 9% after 10 years. Recurrent EAPs were all secreting and 38% provoked clinical symptoms. New lesions were smaller than the primary EAP (P = .01) and more often associated with lymph node metastases (43% vs 4%, P = .01). Operative excision seemed complete in 5 patients. Clinical remission was maintained in 4 patients after a median follow-up of 57 months (IQR, 22-102).


CONCLUSIONS
Local-regional recurrence of sporadic and syndromic EAPs is frequent and may be delayed beyond 10 years, requiring lifelong follow-up after the initial operation. When technically feasible, operative excision can lead to prolonged remission.
Van Slycke
Sam
S
Univ Lille Nord de France, CHULille, Lille, France.
Caiazzo
Robert
R
Pigny
Pascal
P
Cardot-Bauters
Catherine
C
Arnalsteen
Laurent
L
D'Herbomez
Michele
M
Leteurtre
Emmanuelle
E
Rouaix-Emery
Nathalie
N
Ernst
Olivier
O
Huglo
Damien
D
Vantyghem
Marie-Christine
MC
Wemeau
Jean-Louis
JL
Carnaille
Bruno
B
Pattou
François
F
D000328:Adult; D005260:Female; D006801:Humans; D008207:Lymphatic Metastasis; D008297:Male; D008565:Membrane Proteins / Q000235:genetics; D008875:Middle Aged; D009154:Mutation; D009364:Neoplasm Recurrence, Local / Q000209:etiology* / Q000235:genetics / Q000601:surgery; D010236:Paraganglioma, Extra-Adrenal / Q000209:etiology* / Q000235:genetics / Q000556:secondary / Q000601:surgery; D011379:Prognosis; D012086:Reoperation; D012189:Retrospective Studies; D013385:Succinate Dehydrogenase / Q000235:genetics; D013577:Syndrome; D051794:Von Hippel-Lindau Tumor Suppressor Protein / Q000235:genetics; D055815:Young Adult
D008565:Membrane Proteins; C108096:SDHC protein, human; C491545:SDHD protein, human; C108095:SDHB protein, human; D013385:Succinate Dehydrogenase; D051794:Von Hippel-Lindau Tumor Suppressor Protein; C497446:VHL protein, human