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A Child with Malignant Ovarian Tumor and Mullerian Anomally (Mayer Rokitansky Kuster Hauser Syndrome) Getnet Tesfaye * , Bazezew Fekad and Amsalu Worku Bahirdar University, Ethiopia * Corresponding author: Getnet Tesfaye Yihunie, Bahirdar University, Ethiopia, Tel: +251912692839; E-mail: [email protected] Received date: November 11, 2016; Accepted date: January 17, 2017; Published date: January 23, 2017 Copyright: © 2017 Tesfaye G, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Case e patient is a 12 yrs old girl child from an Orthodox family in Amhara which is a very rural area in Ethiopia. She had abdominal swelling since 4 weeks, which was getting worst from the last 2 weeks and having severe persistent abdominal pain. She also had High grade intermitent fever, nausea and vomiting from the last 2 weeks along with urinary urgency, frequency, dysuria and feeling of incomplete voiding. She was under problems like diarhea, constipation, decreased apetite, weight loss, cough, chest pain history of malaria attack, polydypsia, polyphagia family history of Diabetis mellitus and hypertension. Physical Examination General appearance Acutely sick looking in pain BP=100/70 mmHg PR=120/min RR=24/min T=38.4° celcieus HEENT Slightly pale conjuctiva None icteric scelera LGS no Lymphadinopathy Chest clear and resonant Abdomen Distended Abdominopelvic mass extend upto the umblicus Lower border cannot be delinated Tender, irregular border, smooth surface No sign of fluid collection Hypoactive bowel sounds Digital rectal examination Normal anal tone Smooth rectal mucosa ere is smooth tender mass pushing rectal mucosa both inferiorly and posteriorly GUT No visible vaginal bleeding Intact hymen (cruciate type) Patent vaginal canal Digital vaginal examination not done as she is virgin CNS Concious, oriented to time person and place Assesment=Acute abdomen secondary to ovarian cyst torsion en she was admitted to gynecology ward for the following investigation. Investigated with CBC, Stool examination, blood film, Urine analysis Abdomino pelvic Ultrasound ere is 12 × 10 cm echocomplex (cystic with solid appearance) mass on the rt adnexa was noticed. e rt adnexa was looking healthy. Kidneys were present in the renal fossa bilaterally. No hydronephrosis was found. Aſter the physical examinations, she was prepared for laparotomy. Under General anesthesia, patient was cleaned and draped in sterile fashion. Before laparotomy, Informed conscent from father was taken. Infraumblical midline incision Ruptured edge vesicles like solid structure with cystic mass having the size 10 x 8 cm on Rt adnexa, was seen. Lt tubes and ovary were looking healthy (Figures 1-3). Ovarian mass was removed aſter clamping on pedicle and sent for histopathology. Kidneys were explored bilaterally and were given Cſtriaxone 1 gm IV BID & metronidazole 500 mg IV BiD for 48 hrs. Aſter two weeks, the histopathology result came with conclusion of malignant germ cell tumor and she was treated once with BEP (Bleomycin, Etoposide, cisplatin) regimen. Bleomycin 30 mg iv perdose on day 1, 8 and 15 Etoposide 100 mg/m 2 IV per day during day 1 to 5 Cisplatin 20 mg/m 2 IV per day during day 1 to 5 Discussion Congenital Anomalies of the genito-urinary (GU) system are far from uncommon. 10% of infants are born with some genitourinary abnormality [1]. Most are relatively inconsequential. Others can lead to varying degrees of patient morbidity and mortality. e close embryological proximity of the mullerian, wolffian and metanephric systems increases the potential for a common ipsilateral embryological error around the fourth week of gestation. Genital anomalies are four times as common in females as males with unilateral renal agenesis. Uterine anomalies are associated with congenital renal agenesis and Tesfaye et al., J Cancer Diagn 2017, 2:1 DOI: 10.4172/2476-2253.1000109 Case Report Open Access J Cancer Diagn, an open access journal ISSN:2476-2253 Volume 2 • Issue 1 • 1000109 Journal of Cancer Diagnosis J o u r n a l o f C a n c e r D i a g n o s i s

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Page 1: a l ia Jo u rn gnois Journal of Cancer Diagnosis s · A Child with Malignant Ovarian Tumor and Mullerian Anomally (Mayer Rokitansky Kuster Hauser Syndrome) Getnet Tesfaye *, Bazezew

A Child with Malignant Ovarian Tumor and Mullerian Anomally (MayerRokitansky Kuster Hauser Syndrome)Getnet Tesfaye*, Bazezew Fekad and Amsalu Worku

Bahirdar University, Ethiopia*Corresponding author: Getnet Tesfaye Yihunie, Bahirdar University, Ethiopia, Tel: +251912692839; E-mail: [email protected]

Received date: November 11, 2016; Accepted date: January 17, 2017; Published date: January 23, 2017

Copyright: © 2017 Tesfaye G, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricteduse, distribution, and reproduction in any medium, provided the original author and source are credited.

CaseThe patient is a 12 yrs old girl child from an Orthodox family in

Amhara which is a very rural area in Ethiopia. She had abdominalswelling since 4 weeks, which was getting worst from the last 2 weeksand having severe persistent abdominal pain. She also had High gradeintermitent fever, nausea and vomiting from the last 2 weeks alongwith urinary urgency, frequency, dysuria and feeling of incompletevoiding. She was under problems like diarhea, constipation, decreasedapetite, weight loss, cough, chest pain history of malaria attack,polydypsia, polyphagia family history of Diabetis mellitus andhypertension.

Physical ExaminationGeneral appearance

• Acutely sick looking in pain• BP=100/70 mmHg• PR=120/min• RR=24/min• T=38.4° celcieus

HEENT

• Slightly pale conjuctiva• None icteric scelera• LGS no Lymphadinopathy• Chest clear and resonant

Abdomen

• Distended• Abdominopelvic mass extend upto the umblicus• Lower border cannot be delinated• Tender, irregular border, smooth surface• No sign of fluid collection• Hypoactive bowel sounds

Digital rectal examination

• Normal anal tone• Smooth rectal mucosa• There is smooth tender mass pushing rectal mucosa both inferiorly

and posteriorly

GUT

• No visible vaginal bleeding• Intact hymen (cruciate type)• Patent vaginal canal

• Digital vaginal examination not done as she is virgin

CNS

• Concious, oriented to time person and place• Assesment=Acute abdomen secondary to ovarian cyst torsion

Then she was admitted to gynecology ward for the followinginvestigation.

Investigated with

• CBC, Stool examination, blood film, Urine analysis• Abdomino pelvic Ultrasound

There is 12 × 10 cm echocomplex (cystic with solid appearance)mass on the rt adnexa was noticed. The rt adnexa was looking healthy.Kidneys were present in the renal fossa bilaterally. No hydronephrosiswas found.

After the physical examinations, she was prepared for laparotomy.Under General anesthesia, patient was cleaned and draped in sterilefashion. Before laparotomy, Informed conscent from father was taken.

Infraumblical midline incisionRuptured edge vesicles like solid structure with cystic mass having

the size 10 x 8 cm on Rt adnexa, was seen. Lt tubes and ovary werelooking healthy (Figures 1-3). Ovarian mass was removed afterclamping on pedicle and sent for histopathology. Kidneys wereexplored bilaterally and were given Cftriaxone 1 gm IV BID &metronidazole 500 mg IV BiD for 48 hrs.

After two weeks, the histopathology result came with conclusion ofmalignant germ cell tumor and she was treated once with BEP(Bleomycin, Etoposide, cisplatin) regimen.

• Bleomycin 30 mg iv perdose on day 1, 8 and 15• Etoposide 100 mg/m2 IV per day during day 1 to 5• Cisplatin 20 mg/m2 IV per day during day 1 to 5

DiscussionCongenital Anomalies of the genito-urinary (GU) system are far

from uncommon. 10% of infants are born with some genitourinaryabnormality [1]. Most are relatively inconsequential. Others can lead tovarying degrees of patient morbidity and mortality. The closeembryological proximity of the mullerian, wolffian and metanephricsystems increases the potential for a common ipsilateral embryologicalerror around the fourth week of gestation. Genital anomalies are fourtimes as common in females as males with unilateral renal agenesis.Uterine anomalies are associated with congenital renal agenesis and

Tesfaye et al., J Cancer Diagn 2017, 2:1 DOI: 10.4172/2476-2253.1000109

Case Report Open Access

J Cancer Diagn, an open access journalISSN:2476-2253

Volume 2 • Issue 1 • 1000109

Journal of Cancer DiagnosisJour

na

l of Cancer Diagnosis

Page 2: a l ia Jo u rn gnois Journal of Cancer Diagnosis s · A Child with Malignant Ovarian Tumor and Mullerian Anomally (Mayer Rokitansky Kuster Hauser Syndrome) Getnet Tesfaye *, Bazezew

skeletal abnormalities represent an uncommon pathology that oftenpresents important diagnostic and therapeutic problem [2].

Ovarian tumor• Malignant ovarian germ cell tumors (MOGCTs) are rare malignant

tumors that account for about 5% of all ovarian malignancies [3,4]and they usually occur in young females with a peak of incidencebetween 16 and 20 years of age [5].

• The most common histologic type is dysgerminoma followed byimmature teratoma and yolk sac tumor, which together compriseover 90% of all MOGCTs [6,7].

Figure 1: Gross appearance of tumor.

Figure 2: Tumor with healthy looking left ovary.

Figure 3: Tumor ruptured with oozing of vesicle like structure.

References1. Vaughan ED Jr, Middleton GW (1975) Pertinent genitourinary

embryology. Review for the practicing urologist. Urology 6: 139-149.2. Sapienza P, Mingoli A, Noia M, Napoli P, Tallerini A, et al. (1992) Mayer

Rokitansky syndrome. Case report. Minerva Chir 47: 1119-23.3. Smith HO, Berwick M, Verschraegen CF, Wiggins C, Lansing L, et al.

(2006) Incidence and survival rates for female malignant germ celltumors. Obstet Gynecol 107: 1075-85.

4. Jung KW, Won YJ, Kong HJ, Oh CM, Lee DH, et al. (2014) Prediction ofcancer incidence and mortality in Korea, 2014. Cancer Res Treat 46:124-130.

5. Jung KW, Won YJ, Kong HJ, Oh CM, Lee DH, et al. (2014) Cancerstatistics in Korea: incidence, mortality, survival, and prevalence in 2011.Cancer Res Treat 46: 109-23.

6. Abu-Rustum NR, Aghajanian C (1998) Management of malignant germcell tumors of the ovary. Semin Oncol 25: 235-242.

7. Vazquez I, Rustin GJ (2013) Current controversies in the management ofgerm cell ovarian tumours. Curr Opin Oncol 25: 539-45.

Citation: Tesfaye G, Fekad B, Worku A (2017) A Child with Malignant Ovarian Tumor and Mullerian Anomally (Mayer Rokitansky Kuster HauserSyndrome). J Cancer Diagn 2: 109. doi:10.4172/2476-2253.1000109

Page 2 of 2

J Cancer Diagn, an open access journalISSN:2476-2253

Volume 2 • Issue 1 • 1000109