Dr Theresa Raju, Dr Naveen( pediatric surgery), Dr C Jayakumar, Amrita Institute of Medical Sciences, Kochi
Three months old male ba presented to the OPD with complaints of mass the parents in the abdomen which was insidious in onset and progressively increasing in size. Mother also gives history of early satiety. No history of any vomiting or abdominal pain. He was initially evaluated outside for the same
He was admitted for further evaluation and management.
At admission, the child was afebrile. Per abdomen examination showed a large mass per abdomen extending from the epigastrium to below the umbilicus which was tense cystic in consistency. The mass was non tender and there is no warmth. The mass was also mobile transversely with mobility restricted longitudinally. Other systemic examination was within normal limits.
Differential diagnoses
1Ovarian cyst
2. Choledochalcyst
3. Mesenteric cyst
4. Omental cyst
5. Urachal cyst
6. Vestigial remnants
7.Enteric duplication cyst
8. Cystic teratoma
9. Pancreatic pseudocyst
10. Renal cystic disease
11. Splenic cyst
12. Hepatic cyst
Labs: TC:17.09K/UL, N:15.2%, L:75.4%, Hb:10.6g/dl
RFT, LFT, Serum electrolytes did were normal
Beta HCG: <.1Miu/ml, AFP:72.68ng/ml.
Preop serology negative
USG Abdomen was done which showed: a multiseptated cystic intraperitoneal lesion extending from the epigastrium with inferior extent upto level of umbilicus ,extending into subhepatic region.
Craniocaudal extent of 8cm.
Minimal internal vascularity inferiorly,displacing both kidneys posteriorly.
Impression being multiseptated cystic intraperitoneal lesion, Possibility of 1)Cysticlymphangioma 2) Cystic teratoma
Excision of the cyst was done with uneventful post operative period.
Operative findings: Huge cystic lesion with solid area, ? hair within, extending from epigastrium to just above the pelvis. Posterior surface attached to the greater curvature of stomach.
DJ flexure identified posterior to the cyst and whole bowel traced upto sigmoid colon.
Histopathology examination done :showed mature cystic teratoma- no immature/ malignant component.
DISCUSSION:
Mature cystic teratoma is a type of germ cell tumour that contains well differentiated tissues developed from the germ cell layers. Ovarian teratomas are broadly classified histologically into monodermal teratomas, immature teratomas and mature cystic teratoma which is the most common one.
They are often discovered as an incidental findings on radiographic studies during a physical exam or pelvic/abdominal surgery for any other pathology. The most common symptom reported at the time of presentation is lower abdominal pain followed palpable abdominal or pelvic mass.
Some patients present with increasing abdominal girth. Gastrointestinal or urinary symptoms can develop with increasing tumour sie causing compression or invasion of adjacent structures.
Histopath of Teratoma
Mature cystic teratomas are unilocular in 88% of the cases with a cystic cavity lined squamous epithelium. The cyst is predominantly filled with sebaceous material that is liquid at body temperature and solidifies at room temperature. The size of the tumours varies from very tiny cystic masses to as large as greater than 39cm with 80% measuring 10cm or less. These tumours are most frequently unilateral.
On gross appearance, these lesions do not have a characterstic shape or size as it depends on the microscopic heterogenecity of contents. Hair, teeth and bones are easily identifiable on gross examination. Microscopic examination of the contents confirms the diagnosis identifying tissues derived from the following germ cell layers:ectodermal tissue, mesodermal tissue, and endodermal tissue
Evaluation:
Imaging: Although plain radiographs may show radiopaque bodies due to calcifications during work-up for urinary or intestinal pathology, cysts with very little or no calcification can be easily missed. Ultrasound is the preferred initial radiological investigation for the evaluation of adnexal mass. Cystic teratoma appears as a heterogeneous mass with echogenic focus causing acoustic shadowing due to calcification, sebum, and hair. Fat fluid and hair fluid levels are more specific features. Some other peculiar sonographic findings include the Rokitansky nodule, iceberg sign, dot-dash pattern(dermoid mesh), and floating balls sign. Transvaginal ultrasound is superior to abdominal ultrasound for screening for cystic teratomas with a sensitivity of 57.9% and specificity of 99.7%. It has proven to be as accurate as MRI in the identification and characterization of ovarian cystic teratomas.
In torsion, a whirlpool sign with a twisted vascular pedicle is seen on color Doppler ultrasound.Ovarian teratomas can also be overlooked on abdominal ultrasound if it appears similar to bowel gas. CT abdomen and pelvis is believed to be the best modality for evaluation of an adnexal mass, especially for pre-operative assessment
TREATMENT: The primary treatment is surgical excision, The surgery involves complete removal of the cyst to prevent complications such as malignant transformation, rupture or infections
Laparoscopy vs open surgery: Depending on the size and location, the surgery maybe performed using minimally invasive laproscopic approach or through an open surgery if the teratoma is large or complicated
PROGNOSIS:
Prognosis largely depends upon the extent of the disease and its complications. Benign cystic teratomas generally have an excellent prognosis after surgical management with some risk of recurrence in 2 to 10 years. On the contrary cystic teratomas with malignant transformation have variable outcomes depending upon the stage, growth pattern, and vascular invasion of the tumor. According to one study Peterson et al. 75%, the 5-year survival rate was seen in unruptured stage I tumors. Pooled data Kashimura et al. showed a 5-year survival rate in stage I: 50%, 5-year survival rate in stage II: 25%, 5-year survival rate in stage III: 12%, and no survivors in stage IV
TAKE HOME MESSAGE:
Although cystic teratomas are benign tumors, the development of malignancy or complications(torsion, adhesions, rupture, infection), it can rapidly turn into a life-threatening condition with higher morbidity and mortality. Therefore early recognition and appropriate treatment is the most important part of management. A multidisciplinary healthcare team involving surgeon, gynecologist, radiologist, oncologist working together to plan a patient-centered treatment strategy leads to improved patient outcomes. Based on available data, a thorough preoperative risk assessment, surgical cytoreduction, and adjuvant platinum-based therapy with whole pelvic radiation for the early-stage disease have shown some beneficial effects.