Paraganglioma Arising from the Prostate: A CaseReport and Review of the Literature
Yue Chen, Ranlu Liu, Zhihong Zhang, Yong Xu
, Phe
IntroductionParagangliomas are extraadrenal chromaffin tumors. The prepon-
derance of paraganglionic tissue is identified within the adrenal glandmedulla and the extraadrenal paraganglia are distributed throughoutthe body. Extraadrenal paragangliomas can be located anywherefrom the upper neck to the pelvic floor.1 Prostatic paragangliomas areery rare tumors, with no more than 10 cases reported in the litera-ure.2 Because of the nonspecific clinical symptoms, it is difficult to
give a definite diagnosis preoperatively. In this report, we discuss acase of prostatic paraganglioma occurring in a 36-year-old man andthe clinical manifestations, pathologic features, and differential diag-nosis in the context of a review of the literature.
A 36-year-old man complaining of repeated hemospermia formore than 12 months and difficult defecation with bloody stool for 2months was admitted to our hospital; there were no symptoms offrequency, urgency, dysuria, or hematuria. This patient was found tohave a smooth, rubbery enlargement of the prostate on digital rectalexamination, and no median furrow and nodules were palpable. Theserum level of prostate-specific antigen (PSA) was 0.8 ng/mL andother endocrine secretion examinations were normal. B-ultrasonog-raphy showed a 5.3 � 4.6 cm low-echo mass inside the prostate.Magnetic resonance imaging (MRI) indicated a large prostate with a
Yue Chen and Ranlu Liu contributed equally to this work.
Department of Urology, Second Hospital of TianJin Medical University, TianJinInstitute of Urology, Tianjin 300211, China
Submitted: July 21, 2011; Accepted: Sept 8, 2011
Address for correspondence: Yong Xu, MD, NO 23, Pingjiang Road, HeXi District,Tianjin 300211, China
Clinical Pra● Paraganglioma of the prostate is a very rare tumor,
with few cases reported in the worldwide literature.● To our knowledge, our case is the first report of pros-
tatic paraganglioma from China in the literature.● The follow-up is the second longest, ie, � 3 years.● Because of the nonspecific clinical symptoms, it is
difficult to give a definite diagnosis preoperatively.
less uniform signal and a nodule on the right side; the margin of theprostate was discontinuous. There was compression of the adjacentstructures and extension of some prostatic tissue into the bladder.The bladder seminal vesicle triangle was not clear (Figure 1).
Transrectal needle biopsy specimens from the prostate showedthat tumor cells were arranged in nests with eosinophilic cytoplasmand no obvious heteromorphism. The diagnosis before operationwas prostatic sarcoma or carcinoma, but the diagnosis of prostaticparaganglioma could not be excluded, and no estimation of the po-tential of the tumor could be done. Radical retropubic prostatectomywas performed. A big, soft, smooth prostate was found, with partinvading into the bladder and no infiltration. The resected specimenshowed a well-circumscribed tumor involving the bilateral seminalvesicles. The sample section showed a mass measuring 5.0 cm ingreatest dimension, which almost replaced the whole normal pros-tate. The tissue was fleshy and soft with an intact capsule. Necrosisand hemorrhage with exudation of a yellow substance were observedmacroscopically (Figure 2A).
The histologic appearance of the mass indicated that tumor cellswere arranged in either a nest pattern or a cord pattern. The cells wereoval and had medium, lightly stained cytoplasm. Nuclei were largewithout obvious staining. Cytologic atypia or mitosis was absent orrare, although there were some nuclear pleomorphisms. There wasrichly vascularized stroma noted between the cell nests (Figure 2B).The tumor cells were positive for S100 protein and negative for PSAby immunohistochemical staining.
On the basis of histologic and immunohistochemical analysis, thetumor was diagnosed as prostatic paraganglioma.
The patient was discharged on the seventh postoperative day after
ice Points● The purpose of this article is to discuss the
clinical manifestation, pathologic features, anddifferential diagnosis of paraganglioma of theprostate.
● We believe that this study will play an effective role inguiding clinical practice about the diagnosis and ther-apy of prostatic paraganglioma.
for 40 months at last follow-up. Repeated computed tomographicscans as well as MRI showed no lymphadenopathy or recurrentdisease.
DiscussionParagangliomas arise from the embryologic development of the
paraganglia from neural crest cells. Paragangliomas occur most fre-quently in the adrenal medulla, where they are usually referred to aspheochromocytoma; neoplasms derived from paraganglionic tissuein extraadrenal sites are called paragangliomas.3 Extraadrenal pheo-hromocytomas are slow growing, and the majority are located in thebdominal paraaortic region. Paragangliomas of the genitourinaryract can be found in a variety of locations, such as kidney, spermaticord, bladder, urethra, and prostate; in particular, paraganglioma ofhe bladder is not uncommon.4
Prostatic paraganglioma is rare and only a few cases have beenreported in the worldwide literature. The clinical manifestations ofparagangliomas may be highly different, mainly in accordance withthe functional status of the tumor. Because of the compression andobstruction caused by tumors, instead of symptoms related to cate-cholamine excess, the common presentations of nonfunctional para-gangliomas of the prostate are hematuria, hemospermia, and difficulturination and defecation. If the tumor is functional, the clinical man-ifestations result from catecholamine secretion and include hyperten-sion, palpitation, headaches, dizziness, syncope, anxiety, and sweat-ing. These attacks may be precipitated by micturition. The currentcase is an obviously nonfunctional tumor. In the reports about pros-tatic paraganglioma, some of the cases are functional, and their pre-sentations include hypertension, headaches, and sweating.5,6 How-ver hematuria and hemospermia have been the main reportedymptoms in other nonfunctional cases.7,8
The imaging presentations of prostatic paraganglioma on com-puted tomography (CT) or MRI are similar to those of pheochro-mocytoma at other sites. Computed tomographic scans show a solidround or oval mass that is uneven, uniform, or low density. Largerlesions may show a cystic component caused by central hemorrhage
Figure 1 Magnetic Resonance Imaging (MRI) T2-WeightedAxial Image of the Pelvis. MRI Shows a Large Massin the Right Side of the Prostate. There isCompression of the Adjacent Structures. BladderSeminal Vesicle Triangle is not Clear
or necrosis, and calcification is present in some cases. Contrast-en-
hanced pelvic CT is useful for diagnosis, but no computed tomo-graphic feature that is unique for paragangliomas has been found.9
The majority of the tumor margins are clear on pelvic MRI. Themass is usually round, oval, or lobulated with an intact capsule. Thetumors are generally characterized by low T1 and bright T2 signalintensities. Central necrosis is frequently observed. MRI is essentialfor localization, characterization, size, and relationships with adja-cent structures, whereas MRI has important value in differential di-agnosis and detection of extraadrenal paragangliomas.9
Although imaging studies are helpful, the diagnosis of paragangli-oma can be safely accomplished only with histologic and immuno-histochemical evaluation. Histologically, tumor cells of paragangli-oma are characteristically arranged in nests, in acini, or in cords. Thenest cells are surrounded by the argyrophil fibrous network. Tumorcells are polygonal or oval, with medium or plentiful cytoplasm thatmay be lightly stained or eosinophilic. Nuclei are large, with rarenuclear deformity or mitotic activity. The microscopic findings arenot the only predictive proof of malignant potential. The nests arerich in vascularized stroma and thin-walled sinusoids. Immunohis-
Figure 2 Macroscopic and Histologic Performance ofProstatic Paraganglioma. (A) Sectional Profile ofExcised Prostate Specimen. Tissue was Fleshy andSoft With Intact Capsule. Necrosis and HemorrhageCan be Seen Macroscopically. (B) HistologicFindings of Prostatic Paraganglioma (Hematoxylinand Eosin, �100). Tumor Cells are Arranged inNests or in Cords. There is Richly VascularizedStroma
A
B
tochemically, most cases of paragangliomas are strongly positive for
Clinical Genitourinary Cancer March 2012 55
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Paraganglioma Arising from the Prostate: A Case Report and Review of the Literature
56 C
neuron-specific enolase and chromogranin. The sustentacular cellsoften surrounding nests of tumor cells are positive for S100, which isconsistent with our case.
The paragangliomas may be benign or malignant. There is nohistologic criterion to differentiate between benign and malignanttumors; the only evidence for malignancy is the presence of metas-tases. Paraganglioma can metastasize to bone, lung, and lymphnodes.10 Metastases to lymph nodes in prostatic paraganglioma haveeen described in 3 cases.8,11,12 Metastases to other locations haveot been reported. Our case is a benign paraganglioma of the pros-ate. The symptoms and pathologic morphologic characteristics ofost malignant paraganglioma are similar to those of benign tumors;
linical and histologic distinction between benign and malignantases is very difficult.13 Diagnosis of paraganglioma may not be madeasily, in particular because of asymptomatic presentations or non-pecific symptoms.
Because of its rarity in the prostate, paraganglioma may be misdi-gnosed as prostatic adenocarcinoma or sarcoma. When the tumor isarge or exhibits malignant biological behavior, prostatic paragangli-ma may be confused with prostatic sarcoma. They have severaleatures in common, such as enlarged mass, compression of the ad-acent structures, and normal serum levels of PSA and catechol-mine. However the history of prostatic sarcoma is often shorter withapid development and invasive growth. In our case, the preoperativeossibility of sarcoma was considered first in the differential diagno-is. Patients with paragangliomas of the prostate reported in the lit-rature have an average age at diagnosis of 30 years, ranging from 8ears to 75 years. However most cases occur in a relatively youngopulation. Before the diagnosis of prostate adenocarcinoma isiven, the possibility of paraganglioma should be considered in aoung patient.
Surgical resection and radiotherapy are the treatment options foraragangliomas. The former usually includes transurethral resectionf the prostate, radical prostatectomy, or adding pelvic lymphade-ectomy. The latter is used as an analgesic in the patient who has
one metastasis. Additionally, chemotherapy can be considered for
linical Genitourinary Cancer March 2012
atients with metastatic disease.12 Tumor location and size are im-ortant prognostic factors.4 Hence, in order to have a better out-
come, early diagnosis and close follow-up are crucial to detectingrecurrence of paraganglioma.
ConclusionProstatic paraganglioma is uncommon and this case illustrates
the importance of preoperative differential diagnosis. Histologicand immunohistochemical evaluations are necessary for diagno-sis. Surgical excision is the main treatment of choice. Early diag-nosis and close follow-up are crucial to detecting recurrence ofprostatic paraganglioma.
DisclosureThe authors report that they have no relevant relationships to
disclose.
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