Home GUT and OBG A case of Mucinous cystadenocarcinoma of ovary in a 13 year old female
Clinical History
Examination
Investigations
Diagnosis
Treatment
Discussion
References

A 13-year-old nulliparous unmarried female patient hailing from Lucknow presented with non-specific complaints of gaseous distension of abdomen, nausea, and abdominal pain since the past 2 months. There is a history of constipation for the last 1 month which is relieved by laxatives. The consistency, as well as the color of the stools, is normal. The patient also complains of a lump felt in the lower abdomen for the past 1 month which increased in size in the past 1 week. The size of the lump does not vary with cough, posture, or strain. 

Negative History

  • No h/o trauma prior to the swelling
  • No h/o abnormal menstrual flow
  • No h/o significant weight loss or weight gain 
  • No h/o urinary symptoms
  • No h/o similar complaints in the family
  • No h/o past surgeries
  • No h/o use of any contraceptives

Obstetric History

Nulliparous

Age of menarche=13 years

Duration= 5 days/30

Frequency of cycle= 29 days 

No of pads= 2-3/day

Normal flow with no associated pain & clots

General Physical Examination

The patient was alert, conscious, cooperative, and well oriented to time, place, and person. She was moderately built and well-nourished.

Pallor +

No Icterus

No cyanosis

No clubbing

No lymphadenopathy

No edema

Systemic Examination

Per Abdomen- On inspection & palpation, 

Elliptical shaped lump in the right iliac fossa measuring 12×15 cm. The margins are not appreciable. The overlying skin is normal. The size doesn’t increase on cough or strain. No tenderness No pulsatility Compressibility or reducibility is absent. Cystic in consistency

Per Vaginal Examination- On inspection and palpation, vulva is normal. Vaginal walls are normal on 

specular inspection and palpation. CVS- S1, S2 heard. No murmur

RS- B/L Normal vesicular breath sounds, no added sounds

Blood investigations

Hb: 10.70 gm/dl

TLC: 16,200 cells/mm3

Platelet: 6,86,000 cells/mm3

RBC: 3,13,000cells/mm3

MCV- 79.60 fl

MCH- 25.70 Pg

MCHC- 32.20g/dl

RDW-CV- 17.2%

HS CRP- 22.3 mg/L

AFP= 2 IU/ml

Beta HCG= 0.40 mIu/ml

Serum CA-125= 90.9U/ml

Serum CEA= 1.78 ng/ml

CT Scan

A large well-defined space-occupying lesion of size 30x12x10 cm is noted in the left hypochondrium, left lumbar and left iliac fossa region and seen crossing the midline. The solid portion of the SOL shows the presence of calcification & heterogeneous enhancement patterns. Multiple rounded cystic spaces are noted. No significant organ infiltration was noted.

Small bowel loops are displaced to the left side & the uterus is displaced to the right side. 

USG of whole abdomen

A large well defined rounded multiseptated, thick-walled cystic fluid collection/space-occupying lesion of size 19.46 x 20.34 x 19.61 cm & volume 4064 ml is noted filling the entire peritoneal cavity. The cystic space appears clear with few showing bright internal echoes.

Histopathological Examination

Papillary fronds lined by cuboidal to columnar cells with areas of pseudostratification. Individual cells have increased nuclear cytoplasmic ratio and are vesicular to hyperchromatic nuclei. The cells also have intracytoplasmic mucin. Mitotic activity is noted. 

Differential Diagnosis

The following differential diagnosis were considered in view of the age of patient, elevated serum CA-125 level, USG findings, CT findings and histopathological examination.

  • Gastrointestinal Stromal Tumors
  • Dermoid cyst
  • Mature teratoma
  • Sex cord-stromal tumor
  • Serous cystadenocarcinoma
  • Mucinous cystadenocarcinoma

Final Diagnosis

Stage II U/L mucinous cystadenocarcinoma of the left ovary.

Surgery- Left salpingo-oophorectomy with appendectomy was done under general anesthesia.

Chemotherapy cycles were started post-surgery.

Regimen- PACLI CARBO

Inj Paclitaxel 270 mg

In Carboplatin 600 mg

Tab Ondansetron 8 mg SOS 

Tab Domperidone 10 mg TDS for 5 days

Tab Tramadol 50 mg TDS for 2 days

Tab Pantoprazole 40 mg OD for 5 days

Syp Magnesium Hydroxide SOS 

Ovarian tumors are broadly classified into 3 types:

  • Epithelial cell tumors
  • Germ cell tumors
  • Sex cord tumors

Epithelial cell tumors are the most common ovarian tumors overall. In adolescent females, germ cell tumors are most commonly seen. Majority of the ovarian tumors in such an age group are non neoplastic. Therefore, germ cell tumors are usually suspected as the first differential diagnosis in such a patient. Tumor markers are used as part of the preoperative investigation for differential diagnosis in most cases, but they may be important to monitor postoperatively for complete resection of the mass, as well as relapse. CA-125 has the potential to identify epithelial cell tumors while AFP & HCG levels help in identifying Germ Cell Tumors.(Ref 1) In this patient, AFP & HCG levels are normal which are usually elevated in GCTs. Instead, CA-125 levels are elevated which makes us suspect a case of epithelial cell tumor of the ovary. Data shows that the cases of epithelial cell tumors under 20 years of age  are reported in 17% of cases of which only 25% are malignant. (Ref 2)

Epithelial cell tumors are usually b/l and non-malignant  in the majority of cases. (Ref 3) However, in this patient it is u/l and malignant. Surgery is implicated immediately in this case. 

Management of ovarian cysts depends on the patient’s age, size of the cyst, surface invasion and its histo-pathological nature. Conservative surgery as ovarian cystectomy and salpingo-oophorectomy is adequate for benign lesions. However, if there is any sign of malignancy and depending on the stage of the tumor, hysterectomy and debulking surgery along with chemotherapy may also be required. In post menopausal females where this tumor is most commonly seen, debulking surgery with hysterectomy is preferred.

Since this is a case of an adolescent patient, debulking surgery is avoided.

Total Abdominal Hysterectomy with Bilateral Salpingo Oophorectomy is implicated in stage 1 & 2 tumors while Debulking surgery in stage 3 & 4 followed by chemotherapy. (Ref 4)

In our case, although the tumour was removed completely and intact with the affected ovary, our patient was given appointments for 4 cycles of chemotherapy every 3 months for a year. CA-125 level is constantly monitored during this period since there is a chance of recurrence of such a tumor.

  1. Remah M Kamel. A massive Ovarian Cystadenoma. Department of Obstetrics & Gynecology, Faculty of Medicine, Jazan University. December 18, 2009: 3-5
  2. Manoj R Somagutta, Enkhmaa Luvsanyam, Molly S. Jain, Krystel Elliot Thieberge, Amit S. Grewal, Siva K Pendyala, Charles Edwards. A Rare Case of Massive Ovarian Mucinous Cystadenoma With Postmenopausal Bleeding. Cureus. September 02, 2020: 1-2
  3. Şenol Biçer, Zehra Erkul, Necla Peker. A 9-kg Ovarian Mucinous Cystadenoma in a 14-Year-Old Premenarchal Girl. Department of Pediatric Surgery, Erzincan University, Medical School, Erzincan, Turkey. April 22, 2014: 3-7
  4. Baohong Gu, Xuemei Li, Zedong Feng, Yang Yu, Bofang Wang, Maswikiti Ewetse Paul, Hao Chen. A Huge Ovarian Mucinous Cystadenoma in a 15-Year-Old Girl with Situs Inversus Totalis: Report of a Rare Case. July 18, 2020: 2-5

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