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Primary primitive neuroectodermal tumor of the ovary

DOI:10.1016/j.tjog.2013.08.005
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Ling-Hui Chu at Taipei Medical University Hospital
Ling-Hui Chu
Wen-Chun Chang at National Taiwan University
Wen-Chun Chang
Kuan-Ting Kuo
Kuan-Ting Kuo
Kuan-Ting Kuo
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Bor-Ching Sheu
Bor-Ching Sheu
Bor-Ching Sheu
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Research Letter
Primary primitive neuroectodermal tumor of the ovary
Ling-Hui Chu
a
, Wen-Chun Chang
a
, Kuan-Ting Kuo
b
, Bor-Ching Sheu
a
,
c
,
*
a
Department of Obstetrics and Gynecology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
b
Department of Pathology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
c
Centre for Optoelectronic Biomedicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
article info
Article history:
Accepted 27 August 2013
The most common neoplastic ovarian tumor in adolescence is
the germ cell tumor. About 58% of primary ovarian neoplasms are
germ cell tumors, and most are benign cystic teratomas [1]. Ovarian
tumors composed of primitive neuroectodermal elements are
extremely rare.
We report a case of an ovarian primitive neuroectodermal tumor
(PNET) treated with fertility-sparing staging surgery and adjuvant
chemotherapy, followed by six courses of chemotherapy.
A 16-year-old nulliparous girl presented with a pelvic mass
which she had palpated accidentally. The mass grew rapidly over 3
weeks. The patient had experienced intermittent abdominal
discomfort and irregular menstruation in recent months, but there
had been no bowel habit changes. She visited our clinic and her
tumor profile was checked. A detailed sonographic examination
showed one huge, solid, 16.5 cm 9.2 cm pelvic tumor with
heterogenous echo complex contents including some cystic parts
and calcification, with a suspected ovarian origin (Fig. 1). Abdom-
inal and pelvic magnetic resonance imaging revealed a huge mass
lesion in the pelvic cavity, which showed low T1 signal intensity
and intermediate highT2 signal intensity, with some internal cystic
components and good enhancement (Fig. 2).The patient was then
referred to our oncology clinic, where tumor markers for suspected
malignancy revealed an elevated carbohydrate antigen (CA)-125
level (120.9 U/mL), with normal levels of lactate dehydrogenase,
carcinoembryonic antigen, CA-199, alpha-fetoprotein (AFP) and
beta-human chorionic gonadotropin. The patient denied a family
history of ovarian cancer and teratoma. She also denied symptoms
of neuroendocrine activity such as flushing, diarrhea, abdominal
cramping, and palpitations.
An exploratory laparotomy revealed a huge left ovarian solid
tumor with an irregular border and central necrosis (Fig. 3). The
uterus, bilateral fallopian tubes, omentum, and peritoneal surface
were grossly normal. There was a moderate amount of ascites,
about 400 mL. In consideration of her fertility at this young age,
conservative staging surgery with a left salpingo-oophorectomy,
dissection of left side pelvic lymph nodes, and infracolic omental
excision was performed.
Immediate pathological frozen section revealed a spindle cell
tumor. A detailed microscopic pathological examination showed
the well-encapsulated ovarian tumor to be composed of grayish
tan, solid, fleshy tissue with several small cysts with clear fluid.
Marked necrosis was seen.
Microscopically, the tumor showed a high cellularity, composed
of small cells with hyperchromatic, round to oval nuclei and scanty
to small amounts of cytoplasm arranged in lobules separated by
fibrovascular septa, patternless sheets incompletely divided by
fibrovascular septa or a trabecular/cord-like pattern (Fig. 4). A
fibrillary matrix was focally present. Mitotic activity (>10/10 HPF,
high power field) and apoptosis were frequently seen and tumor
necrosis was evident. Immunohistochemically, the tumor was
relatively diffusely positive for synaptophysin and cluster of dif-
ferentiation (CD) 56, focally positive for chromogranin, S-100 and
glial fibrillary acidic protein, with the latter two particularly pre-
dominant in the fibrillary matrix area, but negative for cytokeratin
(AE1/AE3), CD99, and AFP. Based on the above findings, the tumor
resembled a PNET of the central nervous system with a medullo-
blastoma/neuroblastoma pattern. In addition, small areas of mature
teratoma composed of squamous epithelium, respiratory epithe-
lium, bone, cartilage, smooth muscle, adipose tissue, mature glial
tissue, and minor nondescript ducts were also present. Therefore, a
primitive ovarian neuroectodermal tumor in association with a
teratoma was considered.
The patient’s postoperative course was smooth and she was
discharged from the hospital after surgery. Under a diagnosis of left
ovarian PNET, Stage IC, adjuvant chemotherapy for epithelial
ovarian cancer was administered with the PT (carboplatin AUC: 6,
paclitaxel 175 mg/m
2
) protocol six times at 3-week intervals
without severe side effects, except for alopecia.
She was regularly followed for 13 months at our clinic without
evidence of recurrence, with regular menstrual cycles and a normal
*Corresponding author. Department of Obstetrics and Gynecology, National
Taiwan University Hospital, Number 7, Chung-Shan South Road, Taipei 100, Taiwan.
E-mail address: bcsheu@ntu.edu.tw (B.-C. Sheu).
Contents lists available at ScienceDirect
Taiwanese Journal of Obstetrics & Gynecology
journal homepage: www.tjog-online.com
http://dx.doi.org/10.1016/j.tjog.2013.08.005
1028-4559/Copyright ©2014, Taiwan Association of Obstetrics &Gynecology. Published by Elsevier Taiwan LLC. All rights reserved.
Taiwanese Journal of Obstetrics & Gynecology 53 (2014) 409e412
hormone profile. The tumor makers including CA-125 were all
normal during regular monthly follow ups. Follow up with
fluorodeoxyglucose-positron emission tomography 12 months af-
ter completion of chemotherapy also showed negative findings.
Primary neuroectodermal tumors are rare monophasic tera-
tomas composed of immature neuroectodermal tissue. The tumors
are separate from other types of teratomas and the incidence is
rare. These tumors are classified as ependymoma, astrocytoma, and
primitive neuroepithelial tumors such as medulloblastoma,
medulloepithelioma and neuroblastoma [2]. PNETs are highly
cellular and composed of small cells with hyperchromatic, round to
oval nuclei and scanty cytoplasm. Lobules separated by fibrovas-
cular septa are present with prominent areas of necrosis [3].
Based on previous reports, most primary PNETs occur in the
second to third decades of life, at a slightly younger age than the
well-differentiated form of neuroectodermal tumors. The age range
of patients in one study was 13 to 69 years [3]. The prognosis is
generally poor with a high mortality rate.
Kleinman et al [3] reported 12 PNETcases in Stage IA to Stage III.
The duration of posttreatment follow-up ranged from 2 months to
9 years. All patients with Stage IA-IC disease received unilateral
salpingo-oophorectomy. Postoperative adjuvant chemotherapy
was administered in three of four patients with Stage I disease. No
patient had evidence of disease during follow-up. However, nearly
all patients (7 of 8) in Stage III died of disease at 2e20 months after
diagnosis.
Because of the rarity of cases, there is no consensus about the
operative method or adjuvant therapies such as chemotherapy and
radiotherapy. One case report in 2004 described a patient with
PNET Stage IIIC with peritoneal carcinomatosis and extensive
lymphadenopathy who received fertility-sparing staging surgery
and adjuvant radiotherapyand chemotherapy with carboplatin and
paclitaxel. She died after 10 months due to septic shock [4].
The adjuvant chemotherapy regimen varies in reports (Table 1).
Some authors used the bleomycin, etoposide, and cisplatin (BEP)
regimen as in other germ cell tumors, and some used cisplatin,
Fig. 1. Ultrasonography shows a huge, solid, heterogeneous tumor in the pelvic cavity.
Fig. 2. Abdominal and pelvic magnetic resonance imaging reveals a huge mass lesion
with low T1 signal intensity. B ¼urinary bladder; R ¼rectum; V ¼vagina.
L.-H. Chu et al. / Taiwanese Journal of Obstetrics & Gynecology 53 (2014) 409e412410
etoposide, cyclophosphamide, and doxorubicin as a neuroblastoma
treatment protocol [5,6]. In our case, carboplatin and paclitaxel
were used, which followed the regimen for epithelial ovarian
cancer. Megadose chemotherapy followed by peripheral blood
progenitor cell rescue has been reported for metastatic disease [7].
New chemotherapy drugs and drug combinations are being
tested for the treatment of ovarian cancer. Drugs such as tra-
bectedin (Yondelis) and belotecan have shown promise in some
studies. However, we could find no reports on the effects of these
two drugs on ovarian PNET because of the rarity of the disease.
In summary, ovarian PNET is a rare type of germ cell tumor.
The tumor should be differentiated from a wide variety of pri-
mary and metastatic ovarian tumors with similar clinical pic-
tures, and the diagnosis is usually confirmed with pathology.
There is no consensus about the treatment strategy for ovarian
PNET due to the rarity of the disease, although surgery and
chemotherapy are commonly used clinically. More studies are
needed to evaluate the response and effectiveness of different
chemotherapy regimens.
Fig. 3. Left ovarian tumor revealed during exploratory laparotomy (A) and the cut surface (B).
Fig. 4. The tumor shows lobules of small round cells separated by fibrovascular septa
(hematoxylin &eosin stain; original magnification, 400).
Table 1
Cases of primary primitive neuroectodermal tumors (PNET) of ovary mentioning chemotherapy regimens.
Reference Pathology Age (y) Stage Residual tumor after
surgery
Treatment Follow-up recurrence
Demirtas
et al, 2004 [6]
PNET 25 IC Nil Surgery þCT
Left SO, pelvic &para-aortic
lymphadenectomy
BEP 4
VIP 6 (salvage CT)
3 y, NED þ, at lymphocyst
Muhlstein
et al, 2010 [8]
Neuroblastoma 17 IC Nil Surgery þCT
Bilateral SO, omentectomy
cisplatin þetoposide 7
6 y, NED
Ostwal
et al, 2012 [9]
PNET 28 III Nil Surgery þCT
Left SO, cytoreduction,
omentectomy
EFT-2001 protocol
18 mo, DOD þ, pelvis
Lawlor et al, 1997 [7] Neuroblastoma 13 IIIC Diffuse peritoneal
seeding
Surgery þCT
Right SO þomentectomy
Cisplatin þetoposide þ
cyclophosphamide þdoxorubicin
18 mo, NED
Kim et al, 2004 [4] PNET 18 IIIC Diffuse peritoneal
seeding, abdominal
lymphadenopathy
Surgery þCT þRT
RSO, omentectomy, LN biopsy
Carboplatin þpaclitaxel
VACA
10 mo, death
due to septic
shock
þ, para-aortic
LN, femur
Clinkard
et al, 2011 [10]
Medulloblastoma 23 IIIC Diffuse peritoneal
seeding
Bilateral SO, omentectomy
Cisplatin þetoposide
6 y, NED
Ateser
et al, 2007 [11]
PNET 28 IV Lung, adrenal
metastases
RSO
Doxorubicin þ
cyclophosphamide þvincristine
Doxorubicin þactinomycin þ
cyclophosphamide þvincristine
13 mo, DOD þ
BEP ¼bleomycin, etoposide, cisplatin; CT ¼chemotherapy; DOD ¼died of disease; EFT ¼Ewing’s family of tumors; LN ¼lymph nodes; NED ¼no evidence of disease; RT ¼
radiotherapy; SO ¼salpingo-oophorectomy; VACA ¼vincristine, actinomycin, cyclophosphamide, doxorubicin; VIP ¼vinblastine, ifosfamide, cisplatin.
L.-H. Chu et al. / Taiwanese Journal of Obstetrics & Gynecology 53 (2014) 409e412 411
Conflicts of interest
The authors have no conflicts of interest relevant to this article.
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L.-H. Chu et al. / Taiwanese Journal of Obstetrics & Gynecology 53 (2014) 409e412412
... A search of the English-language literature from January 1980 to December 2017 was performed in PubMed, EMBASE and Google Scholar using the following key words: "primitive neuroectodermal tumor", "Ewing's sarcoma" and "neuroectodermal-type tumor". Ultimately, 12 reports comprising 15 cases with detailed clinical data were included for analysis [10][11][12][13][14][15][16][17][18][19][20][21]. ...
... She experienced two spontaneous pregnancies and delivered twice by cesarean section [14]. Another patient, who was 16 years old with stage IC disease, received paclitaxel and carboplatin chemotherapy after primary surgery, and no tumor was detected during the subsequent 13-month follow-up period [21]. The third patient, who was 17 years old with stage IA disease, only accepted left salpingo-oophorectomy and biopsy of the right ovary, but no adjuvant therapy; she remained in CR after a follow-up of 84 months [10]. ...
... Due to the limited sample size, risk factors for survival could not be determined in the present study. Some authors have suggested that the prognosis is poor for those diagnosed with distant metastasis [16,21]. Our analysis also found that even patients with stage I disease may experience rapid relapse. ...
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... With rare exceptions, systemic combination of chemotherapy and definitive local therapy are typically suggested for all patients (6). Grier et al. (8) (9,10). The bleomycin, etoposide, and cisplatin regimen for treating germ cell tumors was also used (11). ...
... Vincristine, ifosfamide, etoposide, doxorubicin, and cyclophosphamide (EFT-2001 protocol, EWS family of tumor protocols) or ifosfamide, epirubicin, and dacarbazine (IAD) can be other alternate regimens for treating other soft tissue sarcomas (12)(13)(14)(15)(16). However, most patients with EFT died within 10 to 18 mo after diagnosis, with the exception of 1 patient with stage I disease (9)(10)(11)(12)(13)(14)(15)(16). ...
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View
... PNETs are uncommon entities especially for the female genital tract and the ovaries are the most common location (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15) . It seems that the exact age for non-skeletal ES is not clear, but cases in the literature were seen between the second and third decades of the life. ...
... The distinction between ES of the ovary and other tumors is made through immunohistochemistry studies. As seen in Table 2, (12)(13)(14)(15)24) on immunohistochemistry, diffuse membranous positivity for MIC2 (CD99), CD56 (neural cell adhesion molecule), HMW CK and FL1 led to the consideration of PNETs. Negativity for epithelial markers such as CK, EMA, desmin, and WT-1 led to the consideration of desmoplastic small round cell tumors (SRCTs). ...
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... The patient age (30 years) respects the characteristic pattern of age distribution encountered in patients with primary ovarian PNET [4,15,16]. A maximum size of 110 mm has also been reported in other scientific articles [3,12], although the dimensions reported in the literature vary from 6.5 cm to 16.5 cm [5,17]. ...
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Kleinman GM, Young RH, Scully RE. Primary neuroectodermal tumors of the ovary. A report of 25 cases
Article
Twenty-five primary ovarian neuroectodermal tumors occurred in females from 6 to 69 (average, 23) years of age; they had the usual presenting symptoms of abdominal swelling or pain. The tumors, which varied from cystic to solid, ranged from 4 to 20 cm (average, 14 cm) in diameter. Microscopic examination revealed three histologic categories--differentiated, primitive, and anaplastic--with the tumors in the first group having a better prognosis than those in the other two groups. Five of the six differentiated gliomas were pure ependymomas, and one was an ependymoma with an astrocytoma component; none contained teratomatous elements. Two patients with stage I tumors were alive 4 and 5 years postoperatively. The one patient with stage IIA tumor was free of disease at 3 years; one of the two patients with a stage III tumor died of tumor after 5 years, and one had two recurrences but was alive and well at 5 years. Twelve tumors were primitive, resembling medulloepithelioma, ependymoblastoma, neuroblastoma or medulloblastoma. Seven tumors had teratomatous foci of other types, including three dermoid cysts. Three patients with stage I tumors were alive at 7 months, 3 years, and 9 years postoperatively; six of seven patients with stage III tumors died of tumor 2 to 20 months postoperatively, and one was alive with disease at 1 year. Seven tumors were anaplastic, resembling glioblastoma. All contained foci of squamous epithelium. One patient with stage IA tumor died of tumor at 2 years, but two were free of tumor after 3 and 4 years. One patient with a stage IIA tumor died of disease after 5 years; another was alive with tumor at 1 year. One patient with a stage III tumor died after 4 months. The differential diagnosis of neuroectodermal tumors of the ovary includes many primary and metastatic ovarian neoplasms of diverse types, and distinction among them is important. Neuroectodermal tumors should be considered when examining unusual ovarian tumors, particularly if the patient is young.
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Metastatic primitive neuroectodermal tumour of the ovary: successful treatment with mega-dose chemotherapy followed by peripheral blood progenitor cell rescue
Article
  • Nov 1997
Primitive neuroectodermal tumours (PNET) of the ovary are rare, aggressive tumours which are associated with high morbidity and mortality. Previously reported cases have shown limited response to therapy in patients presenting with metastatic disease and survival rates have been discouragingly low. We report the case of a 13-year-old girl who presented with a primary ovarian PNET and extensive metastatic disease. Pathologic studies confirmed the neural origin of the tumour and its morphologic appearance of neuroblastoma. Incomplete surgical resection was followed by treatment with aggressive multi-agent chemotherapy including cis-platinum, etoposide, cyclophosphamide, anddoxorubicin as per a neuroblastoma treatment protocol. Complete clinical remission ensued and she received consolidative therapy with myeloablative doses of thiotepa, melphalan, and carboplatin followed by autologous peripheral blood progenitor cell rescue. All therapy was well tolerated and the patient remains in complete remission with no evidence of disease 18 months from presentation. Mega-dose chemotherapy followed by progenitor cell rescue may provide optimal therapy for patients presenting with metastatic ovarian PNET.
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Two successful spontaneous pregnancies in a patient with a primary primitive neuroectodermal tumor of the ovary
Article
To describe a patient with primary primitive neuroectodermal tumor of the ovary with two successful spontaneous pregnancies. Case report. Tertiary center for gynecologic oncology. A 25-year-old woman with two spontaneous pregnancies 5 months after and 2 years after conservative treatment of International Federation of Gynecology and Obstetrics stage IC primary primitive neuroectodermal tumor of the ovary. Assessment of extraovarian spread with staging laparotomy. Four courses of BEP (bleomysin, etoposide, cisplatin) and, for recurrent disease, six courses of salvage VIP (vinblastin, iphosphamide, mesna, cisplatin) chemotherapy. Two successful deliveries and no residual ovarian cancer. A healthy, normal female infant weighing 3600 g was delivered by cesarean section at 38 weeks' gestation. Sixteen months later another infant, a healthy, normal male weighing 3500 g, was delivered by cesarean section at 38 weeks' gestation. No residual cancer was detected at follow-up 12 months after the last delivery. Conservative fertility-preserving treatment might be considered in patients with primary primitive neuroectodermal tumor of the ovary. Without any assisted reproductive technologies, spontaneous pregnancies might occur.
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