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Laparoscopic In-Bag Morcellation 77

5. Sheth SS. An approach to vasicouterine peritoneum through a new About the Author
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Prakash H. Trivedi is one of the
6. Harlow BL, Weiss NS, Lofton S. The epidemiology of sarcomas Pioneer of Endoscopic Surgery
of the uterus. J Natl Cancer Inst. 1986;76(3):399–402. in India and has done more than
35,000 minimal access surgeries.
7. Hilaris GE, Tsoubis T, Konstantopoulos V, Pavlakis K. Feasibility, He was the Past President of
safety, and cost outcomes of laparoscopic management of early (IAGE) and FOGSI. He has
endometrial and cervical malignancy. JSLS. 2009;13(4):489–95. received FOGSI Corion Award
in young category for Hystero-
8. Shen S, Fennessy F, McDannold N. Image-guided thermal therapy scopic TCRE and senior cate-
of uterine fibroids. Semin Ultrasound CT MRI. 2009;30(2):91–104. gory Corion Award for cost-
effective SUI tape in 1995 and
9. Pritts EA, Parker WH, Brown J, et al. Outcome of occult uterine 2005. His research on Fibroid
leiomyosarcoma after surgery for presumed uterine fibroids needs and Fertility received Health
a systematic review. J Minim Invasive Gynecol. 2015;22(1):26–33. Minister Award. He is the Editor
of 8 International books in
10. Mayerhofer K, Obermair A, Windbichler G, et al. Leiomyosar- Gynaecological Advances. His
coma of the uterus: a clinicopathologic multicenter study of 71 centre is recognized by Maharashtra University of Health Sciences in
cases. Gynecol Oncol. 1999;74(2):196–201. the Post-Doctoral Fellowship Course in Minimal Access Gynaecologi-
cal Surgery and FOGSI & ICOG Fellowship course in Gynaecological
11. Quade BJ, Wang TY, Sornberger K, et al. Molecular pathogenesis Endoscopy and Reproductive Medicine which has trained 1500
of uterine smooth muscle tumors from transcriptional profiling. Gynaecs. He was the Scientific Program Chair of AAGL in 2016 and
Genes Chromosom Cancer. 2004;40:97–108. will be the Scientific Program Chair of AAGL in 2020.

12. Pritts EA, Vanness DJ, Olive DL. Prevalence of occult leiomyo-
sarcomas and atypical leiomyomas after laparoscopic morcella-
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2015;12(3):165–77.

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Publisher’s Note Springer Nature remains neutral with regard to
jurisdictional claims in published maps and institutional affiliations.

13

The Journal of Obstetrics and Gynecology of India (January–February 2020) 70(1):78–80
https://doi.org/10.1007/s13224-019-01254-y

CASE REPORT

Pregnancy in a Rare Case of Intracranial Rosai Dorfman Disease (RDD)

Shashikala Ksheerasagar1,2  · N. Venkatesh1 · Niti Raizada1 · K. M. Prathima1 · Ravindra B. Kamble1 · K. Srinivas1 ·
M. A. Suzi Jacklin1 · B. A. Chandramouli1

Received: 11 December 2018 / Accepted: 6 July 2019 / Published online: 2 August 2019
© Federation of Obstetric & Gynecological Societies of India 2019

Introduction cycles previously. She underwent a diagnostic hysterolapa-
roscopy during workup for infertility prior to the IVF cycles.
Rosai–Dorfman disease (RDD) is an uncommon benign Patient had menstrual cycle one month following the last
idiopathic lymphoproliferative disorder. While RDD most chemotherapy after which she had amenorrhea. She was not
commonly affects lymph nodes, extra nodal involvement of on any contraception and had given up hopes of having a
multiple organs has been reported including the CNS. Preg- baby. Ultrasound revealed a single live intrauterine preg-
nancy following chemotherapy for RDD is rather remote. nancy of 7 weeks gestation. Patient continued the pregnancy
Exacerbation of RDD during pregnancy and the need to despite the risk of teratogenicity.
resort to radiotherapy using cyber knife due to aggravation
of symptoms and remarkable recovery thereafter are high- History of RDD
lighted in this case report.
Patient first presented to the neurosurgeon with complaints
Case Report of feeling unsteady while walking which worsened with
movement of head for a duration of 2–3 months. She had
A 38  year-old-gravida 1 married for 12  years, noncon- headache and nasal block since one month, Neurological
sanguineous marriage with history of two failed cycles of examination revealed horizontal gaze dependent nystagmus
invitro fertilization (IVF) 2 years ago, gave history of being bilaterally, decreased hearing in the left ear and tandem
diagnosed with extra nodal Rosai–Dorfman disease (RDD) ataxia. MRI of head and neck showed left petroclival men-
and having undergone six cycles of chemotherapy with ingioma with compression of brain stem. Large mass lesion
cyclophosphamide 1300 mg, Vinblastine 10 mg and pred- in right maxilla, ethmoid, sphenoid sinuses with extension
nisolone 2 months prior to diagnosis of pregnancy. Patient to right orbit and enlarged upper cervical lymph nodes. A
gave history of polycystic ovarian syndrome with irregular differential diagnosis of meningioma/lymphoma/Wegen-
ers Granulomatosis/fungal infection was made. Biopsy
Dr. Shashikala Ksheerasagar is a Consultant Obgyn and of paranasal sinus lesion was advised. Patient underwent
Reproductive Medicine, Vikram Hospital Pvt. Ltd. Dr. N. Septoplasty with right inferior turbinectomy with FESS
Venkatesh is a Consultant Obstetrics and Gynaecology, Vikram (functional endoscopic sinus surgery) and biopsy under
Hospital Pvt. Ltd. Dr. Niti Raizada is a Consultant Medical GA. Histopathology from the lesional tissue revealed dense
Oncologist, Vikram Hospital Pvt. Ltd. Dr. K. M. Prathima is a inflammatory infiltrates with histiocytes showing features
Consultant Pathologist, Vikram Hospital Pvt. Ltd. Dr. Ravindra of Emperipolesis (the presence of an intact cell within the
B. Kamble is a Consultant Radiologist, Vikram Hospital Pvt. Ltd. cytoplasm of another cell) and on immunohistochemistry the
Dr. K. Srinivas is a Consultant Otorhinolaryngologist, Vikram histiocytes were positive for S100 and CD 68. Morphology
Hospital Pvt. Ltd. Dr. M. A. Suzi Jacklin is a Consultant Internal and immunohistochemistry features confirmed the diagnosis
Medicine, Vikram Hospital Pvt. Ltd. Dr. B. A. Chandramouli is a of RDD (Fig. 1).
Consultant Neuro Surgeon, Vikram Hospital Pvt. Ltd.
PET CT scan showed metabolically active lesions in the
* Shashikala Ksheerasagar paranasal sinuses, nasal cavity, spleen, manubrium sternum
[email protected] and cervical lymph nodes. Metabolically active lesions in the
brain -extra axial, infratentorial lesion was noted on the right
1 Vikram Hospital Pvt. Ltd. , Millers Road, Bengaluru 560052, side, extending to the cerebellopontine angle measuring
India

2 Bengaluru, India

1 3Vol:.(1234567890)

Pregnancy in a Rare Case of Intracranial 79

Fig. 1 Histiocytes showing emperipolesis Fig. 2 a Cornal T2W image showing right sinonasal and orbital mass
lesion. b Cornal T2W image showing extradural hypointense mass
45 × 34 mm compressing the cerebellum and pons, 4th ven- lesion in left CP angle
tricle was effaced with no midline shift.
obstruction on the left side. Pediatric surgeon was involved
Chemotherapy was started on patient (CVblP regime and baby underwent colostomy on the 1st neonatal day.
along with premedication and adequate hydration). Repeat Patient had delay in the initiation of lactation but by day 4
PET CT after four cycles of chemotherapy showed partial of the postnatal period lactation was initiated. Two and half
decrease in the size of lesions. In view of partial response to months after delivery patient was admitted for supportive
the therapy, patient was advised two more cycles of chemo- care with antiedema measures as she developed diplopia,
therapy three weeks apart. right sided peri-orbital edema and imbalance while walking.
She was advised stereotactic body radiation therapy by the
At the time of booking visit patient had a BMI of 34.5, medical oncologist. Patient received radical radiation with
hemoglobin of 9.8 gm%. Patient was started on folic acid, cyber knife stereotactic radiation therapy at dose of 20 Gy in
1st trimester screening showed low risk for trisomy 21, 13 five fractions to the residual disease in the right orbital cav-
and 18. However the uterine artery Doppler showed a mean ity and left CP angle lesion with supportive antiedema medi-
PI of 1.7 which is considered to be screen positive for PIH cation over five days. Patient was advised repeat MRI and
as a result of which patient was started on Tab. Ecosprin PET CT after 3 months to evaluate response to treatment.
150 mg at night. Anomaly scan done at 19 weeks and a Post radiation therapy, patient is symptomatically better,
late anomaly scan done at 26 weeks, both revealed mild left there is reduction in cerebellar signs, proptosis and diplopia.
hydronephrosis. Patient had developed GDM by 24 weeks
and was started on Insulin. Patient had difficulty in balancing Discussion
herself in the upright position and found it difficult to walk
without support. Hence MRI was done at 23 weeks gestation RDD is a rare histiocytic disorder and was first described by
which showed a marginal increase in the size of the intrac- Rosai and Dorfman in 1969. It was termed as Sinus histio-
ranial lesion as compared to the PET CT done after the 4th cytosis with massive lymphadenopathy (SHML). It usually
cycle of chemotherapy (Fig. 2). Patient was administered presents with bilateral painless cervical lymphadenopathy,
prophylactic steroids for fetal lung maturity at 32 weeks on fever, night sweats and weight loss. 43% of cases have extra
an inpatient basis as she required blood sugar monitoring. nodal involvement with lesions occurring in the skin, soft
Serial interval growth scans done showed adequate growth tissue, upper respiratory tract, bone, eyes and retro orbital
of the baby and elevated PI of umbilical artery (PI 1.2). tissue. In 22% of cases there is head and neck involvement
The patient’s total weight gain was 8 kgs (BMI was 40.3 @ with nasal cavity being affected more frequently followed by
37 weeks). As her symptoms worsened, decision for elective parotid gland [1], Intracranial RDD is rare and is not well
LSCS under GA was taken at 37 weeks. Spinal anesthesia characterized [2].
was contraindicated due to increased intracranial pressure
and risk of herniation of brainstem. Characteristic histopathology features are histiocytes
showing emperipolesis and immunohistochemistry showed
Elective LSCS was done at 37 + 1 week gestation under
GA, a live male baby weighing 2.69 kg was delivered. Baby
had imperforated anus, atrial septal defect (ASD) and PUJ

13

80 Ksheerasagar et al.

histiocytes positive for S100 and CD 68 and negative for CD1a about teratogenicity and decision to either terminate/continue
[3]. pregnancy was crucial. Symptomatic treatment, managing
blood sugars and timing of delivery with right choice of anes-
Clinical course of RDD is unpredictable with episodes of thesia and multidisciplinary approach all lead to a successful
exacerbation and remission that could last many years. The outcome.
causes of RDD are not fully understood and treatment strate-
gies can differ according to severity or vital organ involvement Compliance with Ethical Standards
[1].
Conflict of interest The authors declare that there is no conflict of inter-
Impact of the Disease on Pregnancy est regarding the publication of this paper and they have not received
any grant.
Though the disease has no direct impact on pregnancy, the Informed Consent Informed consent was obtained from the patient for
chemotherapy received immediately preceding the pregnancy presentation of the case report.
had left its marks on the baby in the form of imperforate anus,
ASD (atrial septal defect), left PUJ obstruction (pelviuretero References
junction). Cyclophosphamide also causes follicular and oocyte
depletion leading to high rates of ovarian failure. In studies 1. Rosai–Dorfman Disease. Histiocyte Society 2011
on mice, cyclophosphamide has shown to be mutagenic to 2. Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, et al. RDD of
preovulatory oocytes.
the central nervous system: report of 6 cases and review of the lit-
Mice oocytes exposed to vinblastine before the first mei- erature. Medicine (Baltimore). 2014;93(3):165–75.
otic division lead to aneuploidy.. The mechanism of action of 3. Dalia S, Sagatys E, Sokol L, Kubal T. RDD: tumor biology,
alkylating agents like cyclophosphamide is by cross-linking clinical features, pathology, and treatment. Cancer Control.
DNA strands, interruption of RNA and protein synthesis and 2014;21(4):322–7.
affecting the cell cycle non-specifically. Vinca alkaloids (Vin- 4. Arnon J, Meirow D, Lewis-Roness H, Ornoy A. Genetic and tera-
blastine) bind tubulin and cause dissociation of the microtubule togenic effects of cancer treatments on gametes and embryos. Hum
apparatus. They act on G1 and S phase of cell cycle only [4]. Reprod Update. 2001;7(4):394–403.
5. Meenu Gill J. ROSAI DORFMAN DISEASE IN A PREGNANT
Impact of Pregnancy on RDD FEMALE: A CASE REPORT - [Internet]. Scopemed.org. 2019.
https://www.scopemed.org/?mno=157043. Cited 22 May 2019
There was worsening of patient’s symptoms with severe imbal- 6. Raychaudhuri R, Hackman S, Norregaard T, Miller D. Intracranial
ance and diplopia as pregnancy progressed. A repeat MRI dur- RDD in pregnancy: a case report. Int J Neuropathol. 2014;2:8–16.
ing the pregnancy showed increase in the size of CNS lesions Publisher’s Note Springer Nature remains neutral with regard to
as compared to the imaging done after four cycles of chemo- jurisdictional claims in published maps and institutional affiliations.
therapy. Patient could not do simple exercises like walking to
help control her blood sugar as she had ataxia. She had prop- About the Author
tosis on the right side and had to undergo an elective LSCS
under GA at 37 weeks as spinal anesthesia was contraindicated Dr. Shashikala Ksheerasa-
in her case. Meenu Gill et al. reported a case of RDD with gar DGO, MRCOG, FRM, is
bilateral massive cervical swelling in a 22-year-old pregnant currently working as consultant
woman. The swelling of the cervical lymph nodes progres- Ob Gyn and Reproductive Medi-
sively increased in size after the onset of pregnancy. FNAC cine at Vikram Hospital, Ben-
diagnosed RDD [5]. galuru. She has worked with Dr.
Kamini Rao, during her fellow-
Raychaudhuri et al. reported a case of intracranial RDD in ship in Reproductive medicine
pregnancy where the patient complained of headache and was from The Rajiv Gandhi Univer-
diagnosed to have a large parafalcine mass following her sec- sity of Health Sciences, Bengal-
ond delivery. Patient underwent neurosurgery and excision of uru. She is a Member of the
the lesion. Histopathology clinched the diagnosis of RDD [6]. RCOG since 2008 and has pre-
sented papers and posters in
Conclusion National and International Con-
ferences. She has also partici-
There is worsening of the condition during pregnancy as seen pated as Faculty in workshops
in our case and also that reported by Meenu Gill et al. Man- and scientific programms conducted by Bangalore Society of Obstret-
aging pregnancy in this case was challenging as counselling rics and Gynaecology and other societies.

13

The Journal of Obstetrics and Gynecology of India (January–February 2020) 70(1):81–85
https://doi.org/10.1007/s13224-019-01243-1

CASE REPORT

Granulosa Cell Tumor of the Ovary Accompanying with Ollier’s Disease:
First Case of Contralateral Presentations

Amirmohsen Jalaeefar1 · Mohammad Shirkhoda1 · Amirsina Shari 2 · Mohsen Sfandbod3

Received: 2 November 2018 / Accepted: 20 May 2019 / Published online: 12 June 2019
© Federation of Obstetric & Gynecological Societies of India 2019

Abstract
Objective Granulosa cell tumor (GCT) is a rare entity of ovarian malignancies. Juvenile GCT is considered a malignant
tumor with an indolent course and tendency toward late recurrence. However, the association of this tumor and multiple
enchondromas has been reported.
Case Presentation A 17-year-old female with abnormal uterine bleeding was referred to our center. Ultrasonographic evalu-
ation revealed a mass with origin in right ovary. Patient was worked up to undergo salpingo-oophorectomy, she felt a dull
pain in her left lower limb. X-ray imaging was indicative for Ollier’s disease at the distal part of femur and proximal part of
tibia. Postoperative pathological review was compatible with juvenile granulosa tumor of the right ovary.
Conclusion This case was the first of its kind that ovarian tumor was contralateral to the side involved by enchondromatosis.

Keywords Granulosa cell tumor · Ollier’s disease · Maffucci syndrome · Multiple enchondromatosis

Introduction epithelial ovarian cancer based on the nature of presentation
and clinical behavior as GCTs occur in younger patients with
Granulosa cell is a somatic cell in the sex cord of the ovary features of hyperestrogenism [4]. The metastatic disease is
playing a role in oocyte development [1]. Granulosa cell usually identified through histopathological study. The bone
tumor (GCT) accounts for 5% of all ovarian malignancies metastasis is very rare [5]. Here we represent a case of GCT
and more than 70% of the sex cord stromal tumors [2]. There accompanying with lower limb chondroma.
are two different histological subtypes of GCT, adult (95%)
and juvenile type (5%) [3]. GCTs can be distinguished from Case Presentation

Amirmohsen Jalaeefar, Assistant Professor at Department of The patient was a 17-year-old female referred to our clinic
Surgical Oncology, Cancer Institute, Tehran University of Medical (Cancer Institute, Tehran University of Medical Sciences,
Sciences, Tehran, Iran; Mohammad Shirkhoda, Assistant Professor Tehran, Iran) with primary diagnosis of an abdominal mass.
at Department of Surgical Oncology, Cancer Institute, Tehran She felt distension of abdomen 3 months prior to the initial
University of Medical Sciences, Tehran, Iran; Amirsina Sharifi, visit at our clinic. An abdominopelvic ultrasonography was
Research Associate at Sina Trauma and Surgery Research Center, advised by a private physician which revealed an abdominal
Tehran University of Medical Sciences, Tehran, Iran; Mohsen mass with probable ovarian origin and ascites.
Sfandbod, Assistant Professor at Department of Hematology and
Oncology, Imam Khomeini Hospital Complex, Tehran University She complained of early satiety, anorexia, menstruation
of Medical Sciences, Tehran, Iran. irregularity and a vague pain in her hip since she felt abdom-
inal distention. On physical examination, a mobile abdomi-
* Amirsina Sharifi nal mass about 20 cm was palpable in the lower abdomen
a.s_sharifi@yahoo.com without any tenderness. On hip examination, mild local ten-
derness was detected on palpation of right iliac wing. There
1 Department of Surgical Oncology, Cancer Institute, Tehran was no other significant sign or symptom in her history or
University of Medical Sciences, Tehran, Iran physical examination.

2 Sina Trauma and Surgery Research Center, Sina Hospital,
Tehran University of Medical Sciences, Hasan Abad Square,
Tehran, Iran

3 Tehran University of Medical Sciences, Tehran, Iran

1 3Vol.:(0123456789)

82 Jalaeefar et al.

Fig. 1 Pelvic magnetic resonance imaging (MRI). A mass arising
probably from right ovary with some necrotic area in the central part

Fig. 3 Left tibia showing multiple radiolucent defects because of
multiple enchondromas (arrow)

Fig. 2 Pelvic X-ray imaging of the patient. Multiple lytic iliac lesions
(arrow)

Abdominopelvic magnetic resonance imaging revealed a Fig. 4 Left femur showing multiple radiolucent defects because of
160 × 80 mm mass with some necrotic areas in the central multiple enchondromas (arrow)
part (Fig. 1). It was seen that the mass originated from the
right adnexa. A lytic lesion also was unveiled in right iliac (IHC was positive for inhibin). Core needle biopsy from
wing suspected to be a metastatic bone lesion. iliac lesion showed normal bone trabeculae. In light of bone
scan and bone biopsy results, we requested plain bone X-ray
Based on MRI result, ovarian tumor markers and from pelvis, skull and her limbs. Multiple lytic bone lesions
whole-body bone scan was requested. The inhibin level were found in iliac wing, distal part of the femur and proxi-
was more than 1000 mcg/dl, and alpha-fetoprotein (AFP), mal part of the tibia. We asked two independent radiologists
carcino-embryogenic antigen (CEA), cancer antigen 125 to interpret lower limb lesions. It was revealed that these
(CA125) and human chorionic gonadotropin (HCG) lev- benign bone lesions were mostly compatible with multiple
els were within normal limits. Other laboratory results chondroma (Figs. 2, 3, 4).
for blood calcium level and alkaline phosphatase were
within normal range. She continued to complain about a The patient underwent laparotomy. A midline incision
dull pain in her left lower limb during diagnostic work up. was made, and 5 L of clear ascitic fluid was evacuated. There
The whole-body bone scan was positive for multiple bone was no evidence of either peritoneal or visceral metasta-
lesions in the distal part of the left femur, the proximal sis. A 15 × 10 cm mass originating from right ovary was
part of the left tibia and the right iliac bone reporting pos- visible which had turned around its pedicle (Fig. 5). After
sible metastatic lesions. right oophorectomy and omentectomy, a piece of the normal

In order to elucidate the definite diagnosis, the multi-
disciplinary team (consisted of gynecologists and obstetri-
cians and oncology surgeons) suggested to take biopsies of
abdominal mass and iliac bone. Abdominal mass core nee-
dle biopsy reported histological features compatible with
ovarian sex cord stromal tumor, especially juvenile GCT

13

Granulosa Cell Tumor of the Ovary Accompanying 83

appearing tissue of the left ovary was sent for frozen sec-
tion analysis. After confirming that the left ovary was his-
tologically normal, a wedge slice of ovarian tissue was sent
for tissue freeze to secure future fertility. The postoperative
period was uneventful, and she was discharged on the third
postoperative day.

The final pathology result confirmed the diagnosis of
juvenile GCT. Definite tumor size was measured and found
to be 10 × 16 × 11 cm, and the IHC result was positive for
CD99 and CD56. Inhibin and calretinin were positive in
tumor cells.

Cytologic evaluation of ascitic fluid was negative for
malignancy. The omentum and right ovary were tumor free
(FIGO stage 1A).

Discussion

Fig. 5 A mass turned around its pedicle resected from right ovary Ollier’s disease (OD) is a nonhereditary skeletal disorder
(size: 10 ×16 × 11 cm) causing multiple enchondromas which may involve tubular
bones [6]. Maffucci syndrome (MS) is an association of mul-
tiple enchondromatosis with soft tissue hemangiomas [7]. In
OD/MS, disruption in cartilage growth leads to abnormal
presentation of cartilage in metaphyseal ends of long bones
giving rise to multiple endochondromas within the metaphy-
sis [8]. About 25–30% of patients with OD/MS experience
malignant transformation and develop chondrosarcomas [8].

Previous studies have reported that the most common
ovarian malignant tumor associated with OD and MS is

Table 1 Review of juvenile granulosa cell tumor associated with multiple enchondromatosis (inclusion criteria was definite pathological diagno-
sis of JGCT and radiological or pathological proof for multiple chondromatosis)

Nos. Authors Years Age (year) Skeletal Dominant side of Side of JGCT Treatment
abnormality chondroma

1 Grenet [10] 1972 1 OD Unknown Unknown Excision of ovarian tumor

2 Lewis [11] 1973 11 MS Left Left Excision of ovarian tumor

3 Sugiyama [21] 1983 3 OD Left Left LSO

4 Tamini and Bolen [17] 1984 15 OD Right Right RSO

5 Pounder [18] 1985 1 OD Right Right Excision of ovarian tumor

6 Vaz and Turner [12] 1986 8 OD Right Right RSO

7 Velasco-oses [22] 1988 6 OD Right Right RSO

8 Asirvatham [13] 1991 13 OD Left Left Left oophorectoy

9 Le Gall [23] 1991 12 OD Right Right RSO

10 Tanaka [9] 1992 15 MS Left Left LSO

11 Gell [14] 1998 13 OD Right Right RSO

12 Hachi [16] 2002 18 MS Right Right RSO

14 Rietveld [15] 2009 36 OD Left Left LSO

15 Burgetova [20] 2017 22 OD Right Right RSO

16 Current case 2018 17 OD Left Right LSO

JGCT Juvenile granulosa tumor, OD Ollier’s disease, MS Maffucci syndrome, RSO right salpingo-oophorectomy, LSO left salpingo-oophorec-
tomy

13

84 Jalaeefar et al.

GCT [9]. The first documented case of this association dates Compliance with Ethical Standards
back to 1972 by Grenet et al. [9–12]. Table 1 summarizes
known cases of the association of OD and MS with juvenile Conflict of interest All authors declare that there is no conflict of inter-
GCT in the literature. It is believed that this association is far est to disclose.
beyond coincidence and it points to generalized mesodermal Human Participants and/or Animals All parts of Declaration of Hel-
dysplasia, as both juvenile GCT and OD/MS are rare dis- sinki have been applied.
eases and enchondromatosis has been associated with other Informed Consent The patient gave us written consent of her inclusion
congenital dysplastic disease [13, 14]. in the study.

The majority of juvenile GCTs occurred in the first or sec- References
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Granulosa Cell Tumor of the Ovary Accompanying 85

16. Hachi H, Othmany A, Douayri A, et al. Association d’une tumeur About the Author
ovarienne de la granulosa juvénile à un syndrome de Maffucci.
Gynécol Obstét Fertil. 2002;30(9):692–5. Dr. Amirmohsen Jalaeefar studied
general medicine at Shahid
17. Tamimi HK, Bolen JW. Enchondromatosis (Ollier’s disease) and Beheshti University of Medical
ovarian juvenile granulosa cell tumor. A case report and review Sciences (1997–2004) and then
of the literature. Cancer. 1984;53(7):1605–8. he succeeded to enter general
surgery at IRAN University of
18. Pounder DJ, Iyer PV, Davy MLJ. Bilateral juvenile granulosa cell Medical Sciences (2006–2010).
tumours associated with skeletal enchondromas. Aust N Z J Obstet He got his fellowship of surgical
Gynaecol. 1985;25(2):123–6. oncology from Tehran Univer-
sity of Medical Sciences (2011–
19. Prat J, Oncology FCOG. Staging classification for cancer of the 2013). At the beginning of his
ovary, fallopian tube, and peritoneum. Int J Gynecol Obstet. career, he became assistant pro-
2014;124(1):1–5. fessor of surgery at Shahid
Beheshti University of Medical
20. Burgetova A, Matejovsky Z, Zikan M, et al. The association of Sciences which lasted for 4 years
enchondromatosis with malignant transformed chondrosarcoma and then he continued as assis-
and ovarian juvenile granulosa cell tumor (Ollier disease). Taiwan tant professor of surgery at Tehran University of Medical Sciences,
J Obstet Gynecol. 2017;56(2):253–7. Cancer Institute from 2 years ago until now. His main interest and
publication is on the head and neck and the abdominopelvic tumors.
21. Sugiyama M, Kohmoto Y, Miyoshi T, et al. In vivo and in vitro
steroid bio-synthesis by ovarian juvenile granulosa cell tumor
of a girl with Ollier’s disease. Acta Gynaecol Obstet Jpn.
1983;35(2):2185.

22. Velasco-Oses A, AlonsoᒧAlvaro A, BlancoᒧPozo A, et al. Ollier’s
disease associated with ovarian juvenile granulosa cell tumor.
Cancer. 1988;62(1):222–5.

23. Le CG, Bouvier R, Chappuis JP, et al. Ollier’s disease and juvenile
ovarian granulosa tumor. Arch Fr Pediat. 1991;48(2):115–8.

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