Full text of DOI:10.1007/BF03344021

J. Endocrinol. Invest. 25: 373-376, 2002
CASE REPORT
A rare association between malignant mediastinal
seminoma and other malignant neoplasms
M. Appetecchia* and E. Pucci**
*
Service of Endocrinology, Regina Elena Cancer Institute - I.F.O., Rome, and **Department of
Endocrinology, University of Pisa, Pisa, Italy
ABSTRACT. Primary malignant mediastinal semi-
nomas (PMMS) are rare tumors accounting for 1-
or thyroid carcinoma. In this report we describe
the case of a patient affected by PMMS and 12
yr later by a malignant brachial plexus Schwanno-
ma and papillary thyroid carcinoma (PTC). Since
both mediastinal seminoma and Schwannoma
were treated with surgery followed by local ra-
diotherapy, we were not able to ascertain if ei-
ther PTC or Schwannoma had been induced by
radiotherapy or represented a casual neoplastic
association.
6
% of all mediastinal tumors. PMMS mostly af-
fect young men, arising from primordial germ
cells that abnormally migrate from the ectoderm
of the yolk sac to the gonadal region. They are
clinically and biologically distinct from primary
testicular tumors and seem to have a worse
prognosis. Due to the rarity of the disease, the
choice of treatment is a matter of debate. Lite-
rature data do not show any association between
this kind of tumor and malignant Schwannoma
(J. Endocrinol. Invest. 25: 373-376, 2002)
©
2002, Editrice Kurtis
INTRODUCTION
tients with mediastinal seminomas can be very
good if the tumors are diagnosed at an early stage.
As only a few cases of these tumors have been re-
ported, the therapy for this disease is still a matter
of debate.
Primary malignant mediastinal seminomas (PMMS)
are rare tumors, histologically identical to their go-
nadal counterpart. They account for 1-6% of all me-
rd
diastinal tumors (1, 4). Mediastinum is the 3 most
frequent site for malignant germ cell tumors after
testes and retroperitoneum. Although uncommon,
mediastinal germ cell tumors are of particular in-
terest since they mostly affect young men aged 20
to 40 yr. Although primary mediastinal seminomas
in women have also been reported (5, 8); clinical-
ly and biologically distinct from primary testicular
tumors, PMMS have similar histological features (2,
Literature data do not show any association be-
tween this kind of tumor and malignant Schwanno-
ma or thyroid carcinoma. In this report, we describe
the case of a patient affected by primary mediasti-
nal seminoma associated with malignant Schwanno-
ma and papillary thyroid carcinoma (PTC).
CASE REPORT
3
). It is widely believed that PMMS have a worse
prognosis than testicular germ cells tumors and
therefore require specific treatment. However,
many Authors believe that the prognosis of pa-
We report the case of a 48-yr-old man who under-
went our evaluation for the first time in June 1974,
accusing fatigue, dyspnea and dysphagia.
Chest X-ray showed a mass in the mediastinum;
chest CT scan demonstrated the presence of a lar-
ge tumor occupying the anterior part of the medi-
astinum and infiltrating the right lung with a maxi-
mum diameter of about 15 cm. Diagnosis of semi-
noma was obtained by exploratory thoracotomy
and biopsy of the tumor.
Key-words: Mediastinal seminoma, extragonadal germ cell tumors, dif-
ferentiated thyroid carcinoma, malignant Schwannoma, familial multiple
neoplasia and lentiginosis syndromes.
Correspondence: Dr. Maria Luisa Appetecchia, Servizio di Endocrino-
logia, Istituto Oncologico Regina Elena - I.F.O., Via E. Chianesi 53,
0
0144 Roma, Italy.
Abdominal and cerebral CT, testicular ultrasonog-
raphy (US), ꢀ-phetoprotein and ꢁ-human chorionic
gonadotropin were within the normal range. The
E-mail: Appetecchia @ifo.it
Accepted October 1, 2001.
373

Mediastinal seminoma and other neoplasms
patient was treated with radiotherapy and 2 months
later a chest CT revealed a complete disappear-
ance of the tumor mass. In October 1986, the pa-
tient referred pain in the left superior limb. A
brachial plexus CT revealed an irregular mass close
to the 1st left rib, behind the succlavian artery. The
patient underwent surgical resection of a tumor
mass of about 6.5 cm of maximum diameter, which
was histologically diagnosed as a malignant Sch-
wannoma, located in the center of the nervous
trunk. The surgical resection was followed by local
radiotherapy.
doses as low as 20 Gray have been shown to be
curative. Some Authors (9, 16, 17) recommended
surgical resection followed by adjuvant irradiation
for small resectable tumors, but most of them be-
lieve that surgery no longer plays a role in the
definitive treatment of seminomas (16, 17). Sur-
gical debulking of large tumors did not prove to
be of benefit in improving local control. Chemo-
therapy was previously used only in advanced ex-
tragonadal seminomas: now it can be the treat-
ment of choice in some other circumstances (18).
A study by Martini et al. (19) showed that 50% of
patients with seminomas had a survival time of
over 10 yr after resection with or without radiation
therapy. Similar results were obtained by other in-
vestigators (20-22). These reports induced us to
treat our patient with radiotherapy alone.
Neurilemmomas or Schwannomas are the most
common tumors on the paravertebral sulcus and
occur commonly in people aged 20 to 50 yr. They
grow slowly and are usually smaller then 5 cm at
the time of diagnosis. The malignant counterpart
of Schwannomas is rare: they are commonly large,
measuring more than 5 cm in diameter and present
with different symptoms according to the location
and the size of the tumor. They may cause superi-
or vena cava obstruction, Horner’s syndrome, dys-
pnea, dysphagia and hoarseness. The diagnostic
criteria as to which tumors are to be designated as
malignant nerve sheath tumors, like: origin from a
major nerve, evidence of Schwann cells, presence
of S-100 protein in tumor cells and nuclear pal-
isading, is a matter of debate (23). Clinically, these
tumors are aggressive and when resected tend to
recur; their 5-yr survival is 75%. Treatment consists
of surgical resection in stage I and II, adding adju-
vant radiotherapy in the latter. Our patient under-
went surgical treatment and subsequent radiother-
apy according to international protocols.
PTC represent 80-85% of well differentiated thy-
roid cancers (24, 25). Exposure to radiation of the
thyroid gland is the only definitively known risk fac-
tor responsible for the increase of the incidence of
well-differentiated thyroid cancers (26, 27). How-
ever, only a small portion of the total annual cases
of PTC is due to radiation (28); 9% of them could
be related to radiation exposure, meaning that 91%
of cases have no clear risk factor, the latency peri-
od after exposure is at least 3 to 5 yr and there is no
apparent drop-off in the increased risk even 40 yr
after the radiation exposure (29, 30). In adult pa-
tients treated with radiotherapy for malignancies,
there is a drop-off of risk reflecting the importance
of age at the moment of exposure (31). Recently,
the Chernobyl nuclear disaster confirmed these da-
One year later a clinical examination showed the
presence of an anterior neck mass. A US examina-
tion demonstrated the presence of a solid nodule in
the right thyroid lobe of 2.6 cm, which resulted to
be “cold” at scintigraphic scan. A fine needle aspi-
ration biopsy (FNAB) of the nodule followed by cy-
tological evaluation indicated the presence of PTC.
Serum levels of free T3 (FT ), free T4 (FT ), TSH and
3
4
TGAb were within the normal range whereas TG
serum values were above the normal range [133
ng/ml, normal values (n.v.) 0-85 ng/ml]. The patient
underwent total thyroidectomy and histological ex-
amination confirmed the previous FNAB diagnosis.
The primary tumor-regional lymphnodes-distant
metastases (TNM) evaluation was pT2bN0Mx, G2,
St.II. 131-Radioiodine ablation (50 mCi) was per-
formed after the surgical procedure because of the
evidence of locoregional residual thyroid tissue at
post-operative 131-iodine whole body scan (WBS).
The patient has shown no evidence of recurrence to
date, although there is a persistent functional deficit
of the left superior limb due to surgical damage of
axillary and radial nerves.
DISCUSSION
Primary pure seminomas represent approximate-
ly 35% of malignant mediastinal germ cell tumors.
2
0-30% of mediastinal seminomas are asymp-
tomatic when discovered (9). Outcome seems to
be influenced by primary mediastinal involvement
by germ cell tumors (10, 11) or by initial tumor vol-
ume (12) or histology. Previous reports confirmed
the good prognosis of mediastinal seminomas
compared with non-seminoma germ cell tumors
(
13, 14). The treatment involves surgery, radio-
therapy and chemotherapy in various combina-
tions. The principal treatment of pure mediastinal
seminomas is traditionally irradiation. PMMS are
extremely sensitive to radiation and long-term sur-
vivors following radiotherapy have been docu-
mented (15). Thirty-five and forty Gray are the
most commonly used radiation doses, although
374

M. Appetecchia and E. Pucci
1
0. Toner G., Geller N., Shiow-Yun L., Bosl G. Extragonadal
and poor risk nonseminomatous germ cell tumors. Cancer
ta showing a great increase in the appearance of
PTC particularly in children which was not related
to the presence of RET/PTC rearrangement but
strictly dependent on radiation (32-34). According
to our experience, literature data reported on the
association of testicular tumors with skin lesions,
gastrointestinal hamartomatous polyps and a vari-
ety of non-endocrine and endocrine tumors such
as thyroid gland tumors named familial lentiginoses
syndromes (Carney complex, Peutz-Jeghers syn-
drome, Cowden disease and Bannayan-Zonana
syndrome) (35-39). Nevertheless, we did not find
any association in the literature between mediasti-
nal seminoma, thyroid carcinoma and malignant
Schwannoma. (35-39). In conclusion, it is difficult to
verify whether thyroid carcinoma arises, in our pa-
tient, as a consequence of previous radiation ex-
posure or could be part of a familial syndrome in
association with mediastinal seminoma.
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