Multiple lymph node metastases in a boy with primary
testicular carcinoid, despite negative preoperative
Jonas Abrahamssona,*, Lotta Mellandera, Ola Nilssonb, Allan Rubenssonc
aDepartment of Pediatrics, Queen Silvias Children’s Hospital, 416 85 Go ¨teborg, Sweden
bDepartment of Pathology, Sahlgrens University Hospital, 413 45 Go ¨teborg, Sweden
cDepartment of Pediatric Surgery, Queen Silvias Children’s Hospital, 416 85 Go ¨teborg, Sweden
Abstract A testicular tumor in a 12-year-old boy proved to be a carcinoid tumor. An extensive
investigation including a computed tomographic scan of the abdominal and pelvic region as well as both
123I-labeled metaiodobenzylguanidine and111In-coupled octreotide scintigraphy was normal. Because
histopathologic examination of the primary surgical specimen revealed tumor growth in the resection
border of the spermatic vessels, a second operation with unilateral lymph node dissection was
performed. Surprisingly, 3 lymph node metastases were found. No further treatment was given and the
boy is alive without disease 9 years after surgery. This case illustrates that modern scintigraphic
techniques do not always detect carcinoid tumors. Because carcinoids respond poorly to other treatment
modalities, the importance of initial radical surgery including a meticulous examination of regional
lymph nodes is emphasized.
D 2005 Elsevier Inc. All rights reserved.
Primary carcinoid of the testis is a very rare tumor. The
tumors may be pure or part of teratomas. Because carcinoid
tumors do not respond well to neither chemotherapy nor
radiotherapy, an initial thorough evaluation of the extent of
disease is essential to facilitate radical surgical removal. We
here describe a primary carcinoid in the testis of a 12-year-
old boy in whom metastatic disease in the retroperitoneal
lymph nodes was present, despite the fact that all imaging
procedures performed were negative.
1. Case report
A previously healthy 12-year-old boy presented with a
firm enlarged right testis. Apart from slight pain from his
right groin, he had experienced no other symptoms.
Ultrasound scan showed a homogeneous tumor. Chest
x-ray and blood levels of alpha-fetoprotein and human
choriogonadotropin were normal. Right inguinal orchid-
ectomy was performed. At surgery, the spermatic cord
appeared thickened and fragile, but repeated biopsies
with frozen sections revealed only inflammatory cells and
A few days after surgery, the pathological report
indicated a carcinoid tumor confined to the testis. Therefore,
0022-3468/$ – see front matter D 2005 Elsevier Inc. All rights reserved.
T Corresponding author. Tel.: +46 31 3434610; fax: +46 31 215486.
E-mail address: email@example.com (J. Abrahamsson).
Journal of Pediatric Surgery (2005) 40, E19–E21
a gastrointestinal series, a barium enema, and computed
tomographic scans of the chest, abdomen, and pelvic region
were performed as well as an123I-labeled metaiodobenzyl-
guanidine (MIBG) and an111In-coupled octreotide scintig-
raphy. All of these investigations were normal as was the
24-hour urinary excretion of 5-hydroxyindoleacetic acid
(5-HIAA). However, careful reexamination of the prepara-
tions from the spermatic cord showed that tumor cells were
present adjacent to the spermatic vessels extending to the
resection border. He was therefore subjected to a retroper-
itoneal lymph node dissection on the right side with
complete removal of the spermatic cord. The pathological
investigation showed carcinoid tumor cells in 3 lymph
nodes and in an additional 5 cm in the perivascular
structures of the distal part of the spermatic cord. He has
subsequently been without signs of disease for a period of
84 months and has been followed up with clinical
evaluation and magnetic resonance imaging of the abdomen
and pelvic region, initially every 3 months.
1.1. Histopathologic findings
Morphological examination of surgical specimens from
the testis demonstrated growth of a typical carcinoid tumor.
Tumor cells grew invasively in the testis, forming nests and
cords. Mitoses were rare and tumor necroses were absent.
Immunocytochemical stainings demonstrated that tumor
cells were strongly positive for general endocrine markers
such as chromogranin A, synaptophysin, and SV2. Tumor
cells were also strongly positive for serotonin (5-HT) and
VMAT1 (Fig. 1). The tumor was therefore classified as a
well-differentiated neuroendocrine carcinoma–malignant
midgut carcinoid .
Carcinoid tumors of the testis are exceedingly rare. In a
study of 7000 testicular tumors, 0.23% were carcinoids .
Patients are usually between 20 and 75 years old and only a
few cases in adolescents have been described . Although
several chemotherapy regimens have been tested in
metastatic carcinoid tumors, including combinations of
5-fluorouracil, streptozocin, and interferon-a, results have
been disappointing [4,5]. Therefore, the mainstay of
treatment of these tumors is radical surgical removal of
the primary tumor and regional metastases.
Because carcinoids are so rare, they usually are not
considered in the initial evaluation of testicular tumors in
children. Most carcinoid tumors in the testis are localized
and patients have an excellent prognosis after surgical
removal. However, metastatic disease has been described
 and the testicular tumor itself may be secondary
mainly to a gastrointestinal primary tumor. Thus, a
meticulous search after an unknown primary or metastatic
disease has to be pursued after orchidectomy. A gastro-
intestinal primary must be excluded through a gastroin-
testinal series and a barium enema. We recommend that
both123I-MIBG scan, which has been shown to detect up
to 85% of known residual carcinoid tumors, as well as
scintigraphy using the somatostatin analogue octreotide
coupled with111In  be used in the diagnostic workup.
transporter 1 (VMAT1) in carcinoid tumor of the testis. Most of the tumor cells are strongly positive for all markers with a granular
cytoplasmic staining pattern. Immunoperoxidase staining of paraffin sections.
Immunocytochemical demonstration of chromogranin A (CgA), synaptophysin (SYN), serotonin (5-HT), and vesicular monoamine
J. Abrahamsson et al.E20
Furthermore, MRT imaging of the retroperitoneal and Download full-text
pelvic region should be performed.
However, in our patient, none of these investigations
revealed the presence of the retroperitoneal lymph node
metastases. Furthermore, as is often the case in primary
carcinoids of the testis, urinary excretion of 5-HIAA was
normal. The histological preparations showed that the tumor
extended along the outer wall of the spermatic vein, but the
assessment of disease extension was complicated by the fact
that the tumor did not grow continuously along the
Thus, although the above-mentioned imaging procedures
are highly sensitive in detecting carcinoid tumors, we
recommend that when tumor growth is demonstrated in the
the affected spermatic cord as well as a unilateral lymph node
dissection, should be undertaken. It has been suggested that
intraoperative use of a handheld gamma-detecting probe to
detect in situ tumor binding of radioiodinated octreotide can
improve the sensitivity in detecting residual tumor .
In conclusion, our case demonstrates that despite the use
of modern sensitive imaging techniques, residual disease
was not detected. Radical orchidectomy with careful
histological examination of the spermatic cord should be
performed in all patients with carcinoid tumors of the testis.
After primary therapy, careful monitoring of local recur-
rence or metastatic lymph node spread is essential.
 Jakobsen AM, Andersson P, Saglik G, et al. Differential expression of
vesicular monoamine transporter (VMAT) 1 and 2 in gastrointestinal
endocrine tumours. J Pathol 2001;195:463-72.
 Berdjis CC, Mostofi FK. Carcinoid tumors of the testis. J Urol
 Leake J, Levitt G, Ramani P. Primary carcinoid of the testin in a 10-
year old boy. Histopathology 1991;19:373-5.
 Kvols LK, Buck M. Chemotherapy of metastatic carcinoid and islet cell
tumors. Am J Med 1987;82:77-83.
 V7lim7ki M, J7rvinen H, Salmela P, et al. Is the treatment of metastatic
carcinoid tumor with interferon not as successful as suggested? Cancer
 Kaufman JJ, Waisman J. Primary carcinoid tumour of testis with
metastasis. Urology 1985;25:534-6.
 Kwekkeboom DJ, Krenning EP, Bakker WH, et al. Somatostatin
analogue scintigraphy in carcinoid tumours. Eur J Nucl Med
 Schirmer WJ, O’Dorisio TM, Schirmer TP, et al. Intraoperative
localization of neuroendocrine tumors with 125I-TYR(3)-octreotide
and a hand-held gamma-detecting probe. Surgery 1993;114:745-51.
Multiple lymph node metastases in a boy with primary testicular carcinoidE21