|Year : 2017 | Volume
| Issue : 4 | Page : 224-226
Primary retroperitoneal yolk sac tumor
KS Sahana1, RM Prakash Saldanha1, Guruprasad Bhat2
1 Department of Paediatrics, Yenepoya Medical College and University, Mangalore, Karnataka, India
2 Department of Medical Oncology, Yenepoya Medical College and University, Mangalore, Karnataka, India
|Date of Web Publication||5-Feb-2018|
K S Sahana
Department of Paediatrics, Yenepoya Medical College and University, Deralakatte, Mangalore - 575 018, Karnataka
Source of Support: None, Conflict of Interest: None
Primary retroperitoneal yolk sac tumour is very rare. 3 year old with rapidly progressing abdominal mass. CT heterogeneously enhancing large lesion with liver metastasis. HPE showed features suggestive of YST. Elevated AFP noted. Patient improved with surgery followed by chemotherapy. Paediatric germ cell tumours are highly curable form of cancer, but their diversity makes it challenging for the clinicians.
Keywords: Primary, retroperitoneal, Yolk sac tumour
|How to cite this article:|
Sahana K S, Prakash Saldanha R M, Bhat G. Primary retroperitoneal yolk sac tumor. Apollo Med 2017;14:224-6
| Introduction|| |
Malignant germ cell tumors (MGCTs) are rare, with an incidence of 12 cases/1 million. MGCTs account for approximately 2%–3% of cancer cases in children and adolescents younger than 15 years, but incidence increases during adolescent period (16%).
Yolk sac tumors (YSTs) occur typically in gonads (testis and ovaries). Extragondal YSTs are very rare accounting for only 1%–5% though it is seen more often in children <15 years of age. The most common extragonadal sites are sacrococcygeal, mediastinal, intracranial, and retroperitoneal. The majority of retroperitoneal GCTs in male patients represent a metastasis of a testicular primary lesion. Exclusively primary retroperitoneal is uncommon, comprising <5% of all extragonadal GCTs. Till now, very few cases of primary retroperitoneal tumor cases have been reported in the literature. These facts make this case report very interesting as the site of presentation is very rare.
| Case Report|| |
A 3-year-old child presented with a history of abdominal distension since 1 week. It was insidious in onset and rapidly progressive. There was no history of fever, loose stool, abdominal pain, vomiting, weight loss, or loss of appetite.
On examination, child weight and height were <3rd percentile according to WHO charts. Vitals were normal. Pallor was present on general physical examination. On per abdomen examination, a single smooth hard mass measuring 12 cm × 10 cm was present in the umbilical region extending from the epigastric region to the left lumbar region and right hypochondrium. There was no organomegaly, or there was no evidence of free fluid. Testes were normal. Other systems were within normal limits.
Investigations showed hemoglobin of 7.8 g/dl, total count of 16.5/cumm, and platelet count of 4.55 lacks. Renal function test, liver function test, prothrombin time, activated partial thromboplastin time, and serum electrolytes were within normal limits. Ultrasonography (USG) abdomen showed a large mixed mass noted in epigastrium measuring 9.4 cm × 9.3 cm behind stomach abutting body and tail of pancreas with cystic areas, suggestive of pancreatic lesion or an adrenal cyst. Computed tomography scan showed heterogeneously enhancing large 10 cm × 8.8 cm × 10 cm well-defined lesion seen in the epigastric region extending from the subhepatic region up to the level of the umbilicus. Multiple hypodense lesions noted in both lobes of the liver, largest in right lobe (1 cm × 1 cm) [Figure 1]. The above features were suggestive of mesenteric/mesenchymal tumor or primitive neuroectodermal tumor with liver metastasis. Alpha-fetoprotein (AFP), beta human chorionic gonadotropin, and lactate dehydrogenase (LDH) were sent which showed the elevation of AFP (7285 IU/ml) and LDH (1777 u/L).
|Figure 1: Computed tomography abdomen showing the extent and nature of the tumor|
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Exploratory laparotomy showed a spheroidal tumor arising from the retroperitoneum in the lesser sac abutting posterior wall of stomach superiorly and transverse colon inferiorly and wrapped by omentum measuring 15 cm × 15 cm. Retroperitoneal tumor excised and was sent for histopathological examination which showed cells arranged in microcystic glandular and reticular pattern. Cells were cuboidal with high nuclear-cytoplasmic ratio and hyperchromatic nuclei with atypical mitotic figures. Schiller-Duval Bodies More Details and hyaline globules were also noted suggestive of Endodermal sinus tumor [Figure 2].
|Figure 2: Schiller-Duval bodies and hyaline globules noted within the tumor tissue|
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Three days later, patient developed small bowel obstruction and was taken up for exploratory laparotomy. Obstruction was released by excising the gangrenous transverse colon, and end-to-end anastomoses were done. Later, he developed a fecal fistula which was conservatively managed. By this time, liver rapidly increased in size due to metastasis. For further management, patient was referred to medical oncology. Chemotherapy was started with following drugs: injection etoposide, injection cisplatin, and injection bleomycin. Child received four cycles of chemotherapy at a 3-week interval. At the end of 4 weeks of chemotherapy, child was asymptotic and AFP level decreased to 2 IU/ml.
| Discussion|| |
Retroperitoneal tumors are one of the important group of tumors of pediatric age group and commonly seen retroperitoneal tumors are neuroblastoma, Wilms tumor, and teratomas. The GCTs develop from primordial germ cells of the embryo which originate from the yolk sac endoderm. They migrate around the hindgut to the genital ridge on the posterior abdominal wall and become part of the developing gonad and produce ova or the sperm. GCTs are the tumors which show diversity in their clinical presentation and histopathological features. Five major types seen are teratoma, YST, germinoma, embryonal carcinoma, and choriocarcinoma. First two are seen more commonly in pediatric age group. Teratoma is the most common variety with benign (mature) or immature histological features. Ovarian cysts are frequently encountered in female newborns, but most of them are benign and self-limiting except malignant cystic teratomas.
In general, 80% of GCTs are benign, while only 20% are malignant (representing about 2%–3% of all malignant pediatric tumors). YST, also known as endodermal sinus tumor, is the most common pure malignant GCT in young children.
The histopathological features of YST are distinctive, with the proliferation of tubular and papillary structures and sinusoidal formation form fibrovascular cores lined by tumor cells (Schiller-Duval bodies) with frequent mitotic figures. Periodic acid–Schiff-positive hyaline globules can be identified.
Serum AFP level and LDH will be elevated. Serum AFP is one of the hallmarks of YST and facilitates its diagnosis. The serial measurement serum AFP is useful for monitoring its clinical course and response to treatment.
Diagnosis begins with physical examination and imaging studies including plain radiographs of the chest and USG of the abdomen. Complete surgical excision of the tumor usually is indicated, except for patients with intracranial tumors, followed by chemotherapy. The overall cure rate for children with GCTs is >95%. Before the advent of combination chemotherapy, the prognosis for patients with YST was poor, with an 80%–90% mortality rate within 2 years of diagnosis. Combination of bleomycin, etoposide, and cisplatin was demonstrated to be highly active against MGCT and is the standard treatment for this type of tumor which is given for 3–4 cycles depending on the stage of the tumor., Patients with metastasis secondary to hematogenous spread have also been treated successfully with chemotherapy as in our case.
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 initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Ashraf Ahamad, Associate professor, Department of Paediatric Surgery, Yenepoya University for his contribution in managing the case.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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