Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2022  |  Volume : 27  |  Issue : 3  |  Page : 365--367

Yolk Sac Tumor of the Cervix in an Infant: A Surgical Dilemma


Swati Narasimhan1, Janani Arul2, Rajendran Swaminathan3, Prakash Agarwal4,  
1 Department of Paediatrics, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
2 Department of Paediatric Haematology and Oncology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
3 Department of Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
4 Department of Paediatric Surgery, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Correspondence Address:
Dr. Janani Arul
A3-301, Purva Windermere Surya nagar, Pallikaranai, Chennai - 600 100, Tamil Nadu
India

Abstract

A 5-month-old child presented with vaginal bleeding and found yolk sac tumor of the uterine cervix. She attained complete remission only with chemotherapy and on follow-up for more than a year. We report this for the unusual age and feasibility of avoiding surgery in selected cases.



How to cite this article:
Narasimhan S, Arul J, Swaminathan R, Agarwal P. Yolk Sac Tumor of the Cervix in an Infant: A Surgical Dilemma.J Indian Assoc Pediatr Surg 2022;27:365-367


How to cite this URL:
Narasimhan S, Arul J, Swaminathan R, Agarwal P. Yolk Sac Tumor of the Cervix in an Infant: A Surgical Dilemma. J Indian Assoc Pediatr Surg [serial online] 2022 [cited 2022 Jun 25 ];27:365-367
Available from: https://www.jiaps.com/text.asp?2022/27/3/365/345137


Full Text



 Introduction



Yolk sac tumors (YSTs), also known as endodermal sinus tumors (ESTs), are the most common malignant germ cell tumors (GCTs) with classically elevated alpha-fetoprotein (αFP). Gonadal GCTs are common, but only 10%–15% occur in extragonadal sites. The genital location comprises 3%–4% of all GCTs (commonly ovary, followed by the vagina, cervix, and vulva).[1] Diagnosis by biopsy is warranted to differentiate this from the more common embryonal rhabdomyosarcoma, botryoid subtype when it occurs in vagina or cervix.

Surgery has played an important role in the treatment of malignant GCTs since 1965. However, surgical option in the extragonadal sites can be mutilating affecting fertility.[2] We report an infant with GCT of the cervix which was treated with only chemotherapy and in remission for more than a year.

 Case Report



A 5-month-old female infant, firstborn to nonconsanguineous married parents, presented with intermittent bloody discharge in her diapers for the past 3 weeks. The mother's antenatal history was uneventful. The baby was born by normal vaginal delivery. Postnatal and early infancy period was uneventful with developmental milestones appropriate for age. She had no facial dysmorphism or any congenital anomalies. Her systemic examination was normal. Pelvic ultrasonography revealed a well-defined mass in the uterus measuring 3.0 cm × 3.0 cm × 2.5 cm. Hence, magnetic resonance imaging pelvis done showed a heterogeneous and hyperintense mass measuring 3.9 cm × 2.4 cm × 2.8 cm arising from the cervix and lower uterine body, posterior to the bladder [Figure 1]. Blood investigations were normal, except for elevated serum αFP (12,587 ng/mL). To rule out metastasis and to understand the complexity of the tumor location, whole-body positron emission tomography (PET) Laparoscopic biopsy of the lesion revealed atypical cells, arranged in glands and microcystic pattern with mild nuclear pleomorphism. Immunohistochemistry was positive for cytoplasmic glypican 3 and negative for vimentin, desmin, and SMA, confirming the diagnosis of YST (EST) [Figure 2].{Figure 1}{Figure 2}

The family was counseled regarding the diagnosis and instituted with (etoposide, carboplatin, and bleomycin (JEB) chemotherapy with appropriate supportive measures. The possibility of surgical excision of the tumor mass along with hysterectomy was informed to them. Parents were unwilling for surgical excision even if there was a residual mass after chemotherapy. Hence, she received 6 cycles of JEB chemotherapy. Serial AFPs showed decreasing trend; post 6 cycles chemotherapy, AFP was 18 ng/mL. The multidisciplinary tumor board meeting suggested PET-CT as the mode of imaging following completion of the surgery, to rule out any residual disease. As it showed complete response, surgery was deferred. Currently, she is on 6 monthly follow-up for the past 18 months and is in complete remission (last AFP 5 ng/mL).

 Discussion



Vaginal bleeding in an infant is not so common, and even when a clinician encounters this, GCTs are not considered usually in the differential diagnosis. Although GCTs arising in the pelvis outside the ovary are extremely rare, extragonadal GCTs in the cervix, vagina, endometrium, and retroperitoneum have been cited and failure to consider can cause significant delay, thereby increasing the mortality, especially at this very young age.[3]

Due to rarity of this malignancy, particularly in very young infants, an internationally standard treatment protocol is not available. As the YSTs are locally aggressive, the standard treatment option has been surgery (subtotal excision, total hysterectomy) with adjuvant chemotherapy. Recently, literature has demonstrated a 4-year survival in 90% of cases following only chemotherapy. Similar studies by Yuan et al. and Tao et al. have shown infants with vaginal YST who achieved completed remission, with only chemotherapy without surgery.[4],[5]

The awareness and knowledge about these rare tumors are essential to identify them at an early stage. As there is a paucity of data in treating cervical and uterine GCTs, a collaborative approach with surgeons and pediatric oncologists is needed in making the treatment protocol addressing the surgery depending on the residual tumor postchemotherapy. Treatment with chemotherapy alone or in combination with organ-sparing surgical resection may allow preservation of later sexual function and fertility if there is no residual tumor postchemotherapy. However, larger studies are needed in international level with institutions across the countries considering the rarity of this tumor at this age.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgment

We would like to Dr. Julius Scott (Professor, Paediatric Hematology and Oncology, Sri Ramachandra Hospital) for sharing his knowledge and giving inputs to this case report.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Billmire DF. Malignant germ cell tumors in childhood. Semin Pediatr Surg 2006;15:30-6.
2Collins HS, Burke TW, Heller PB, Olson TA, Woodward JE, Park RC. Endodermal sinus tumor of the infant vagina treated exclusively by chemotherapy. Obstet Gynecol 1989;73:507-9.
3Chen SJ, Li YW, Tsai WY. Endodermal sinus (yolk sac) tumor of vagina and cervix in an infant. Pediatr Radiol 1993;23:57-8.
4Yuan Z, Cao D, Yang J, Keng S, Huang H. Vaginal yolk sac tumors: Our experiences and results. Int J Gynecol Cancer 2017;27:1489-93.
5Tao T, Yang J, Cao D, Guo L, Chen J, Lang J, et al. Conservative treatment and long-term follow up of endodermal sinus tumor of the vagina. Gynecol Oncol 2012;125:358-61.