Retroperitoneal paragangliomas: role of immunohistochemistry in the diagnosis of malignancy and in assessment of prognosis.
OBJECTIVE
To present our experience of 10 patients with extra-adrenal retroperitoneal paragangliomas, and assess prognostic tests.


METHODS
Retrospective study of casenotes.


METHODS
University hospital, Italy.


METHODS
10 Patients who presented with paragangliomas between 1970 and 1991.


METHODS
Histological and immunohistochemical results, and outcome.


RESULTS
All tumours were completely resected and there was no operative mortality. Of the 8 patients who had no metastases at presentation 3 died of recurrence 3, 5, and 10 years later, respectively; 4 were alive and free of disease 2-7 years after diagnosis. The 2 patients with synchronous bone metastases at presentation died 1 and 4 years later. Immunohistochemical analysis of type I cells (chromogranin A and neurone-specific enolase) showed little correlation with progression of disease, but there was a correlation between the presence of type II cells (S100 protein) and good prognosis.


CONCLUSIONS
Excision is the treatment of choice for paraganglioma. Immunohistochemical techniques may provide useful information about prognosis, in particular about those patients who are at increased risk of recurrence. Long term follow up is essential, because successful management of recurrence is dependent on early recognition.
Montresor
E
E
Department of Surgery, University of Verona, Italy.
Iacono
C
C
Nifosi
F
F
Zanza
A
A
Modena
S
S
Zamboni
G
G
Bernardello
F
F
Serio
G
G
D000328:Adult; D001859:Bone Neoplasms / Q000401:mortality / Q000556:secondary; D053379:Chromogranin A; D002864:Chromogranins / Q000378:metabolism; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000401:mortality; D010236:Paraganglioma, Extra-Adrenal / Q000175:diagnosis / Q000378:metabolism / Q000401:mortality / Q000556:secondary / Q000601:surgery*; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D011183:Postoperative Complications / Q000401:mortality; D011379:Prognosis; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis / Q000378:metabolism / Q000401:mortality / Q000601:surgery*; D012189:Retrospective Studies; D009418:S100 Proteins / Q000378:metabolism; D013514:Surgical Procedures, Operative / Q000379:methods; D013997:Time Factors; D014057:Tomography, X-Ray Computed
D053379:Chromogranin A; D002864:Chromogranins; D009418:S100 Proteins; D010751:Phosphopyruvate Hydratase