Indian Journal of Pathology and Microbiology

: 2021  |  Volume : 64  |  Issue : 4  |  Page : 854--857

Pure gastric yolk sac tumor: A case report and review of literature

Pallavi Srivastava1, Nuzhat Husain1, Saumya Shukla1, Anshuman Pandey2,  
1 Department of Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, Vibhuti Khand, Gomti Nagar, Lucknow, Uttar Pradesh, India
2 Department of Surgical Gastroenterology, Dr Ram Manohar Lohia Institute of Medical Sciences, Vibhuti Khand, Gomti Nagar, Lucknow, Uttar Pradesh, India

Correspondence Address:
Nuzhat Husain
Dr Ram Manohar Lohia Institute of Medical Sciences, Vibhuti Khand, Gomti Nagar, Lucknow - 226 010, Uttar Pradesh

How to cite this article:
Srivastava P, Husain N, Shukla S, Pandey A. Pure gastric yolk sac tumor: A case report and review of literature.Indian J Pathol Microbiol 2021;64:854-857

How to cite this URL:
Srivastava P, Husain N, Shukla S, Pandey A. Pure gastric yolk sac tumor: A case report and review of literature. Indian J Pathol Microbiol [serial online] 2021 [cited 2023 Jan 30 ];64:854-857
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Dear Editor,

Primary germ cell tumors usually arise in the testes or ovaries; however, they also occur in extragonadal regions, including the mediastinum, the retroperitoneum, the sacrococcygeal region, and the intracranial region.[1] Gastric germ cell tumors are among the uncommon histological variant. Most of the gastric yolk sac tumors have poor survival as they are diagnosed late and usually present with wide spread metastasis at the time of diagnosis. To best of our knowledge this is the eighth case of gastric yolk sac tumor.[1],[2],[3],[4],[5],[6],[7] We present a case of 43-year-old female admitted to gastrosurgery department with a medical history of pain in epigastrium, postprandial fullness, and bloating since 4 months along with vomiting, black stools, loss of weight and appetite. Gastroscopy examination revealed a large ulcerated tumor located in the antrum [Figure 1]a, a computed tomographic (CT) scan of the abdomen showed diffuse nodular irregular thickening of distal body pyloric antrum with small extension in the duodenal bulb. Maximum wall thickness was approx. 25 mm. No direct extension to colon or pancreas was seen [Figure 1]b. Enlarged discrete and confluent lymph nodes are seen in the gastrohepatic, portahepatis, and subpyloris region [Figure 1]c. Small nodular deposits are noted in the omentum. Fine-needle aspirate was not performed from the lymph nodes as small tissue biopsies taken during endoscopic evaluation was taken and histological diagnosis of adenocarcinoma was rendered. Laboratory investigations including CEA & CA19.9 having normal values with elevated alkaline phosphatase & SGOT levels. The patient underwent palliative distal gastrectomy with billroth II duodeno-jejunostomy and feeding jejunostomy. A distal gastrectomy came for histopathological examination grossly showing solid white friable tumor measuring 6 × 1.8 cm reaching up to serosal surface. The sections from growth showed variegated appearance with tumor cells arranged in sheets, microcyst, macrocysts filled with eosinophilic secretions along with pseudopapillary structures [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 2]f with schiller-Duval bodies [Figure 2]g and [Figure 2]h. The neoplastic cells have pale eosinophilic to water-clear cytoplasm including some hyaline droplets with round to oval vesicular nuclei and variably sized nucleoli. Pertaining to the large size of mass and unusual morphology about ten sections were taken from different areas of the mass to rule out adenocarcinomatous component in the tumor. IHC was performed largely on sections showing viable tumor areas with adjacent normal gastric epithelium for positive control. The tumor cells showed immunoreactivity for pan cytokeratin [Figure 3]a, SALL 4 [Figure 3]b, α-fetoprotein [Figure 3]c focal positivity for PLAP [Figure 3]d with negative expression for synaptophysin, chromogranin A, cytokeratins 7 and 20, β-HCG, CDX2, CD30 & Hepar 1 [Figure 3]e, [Figure 3]f, [Figure 3]g, [Figure 3]h. Postoperatively α-fetoprotein was 57270 IU/ml (normal range: 0.5-5.5 IU/ml) and a final diagnosis of gastric yolk sac tumor was rendered. The serum α-fetoprotein dropped to 28,000 after one month of surgery however normal values were not reached. The patient further underwent 4 cycles of chemotherapy (Day 1-4 cisplatin 20 mg and etoposide 100 mg). In addition, support with granulocyte stimulating factor was needed because of hematological toxicity. After four cycles of chemotherapy a repeat α-fetoprotein was done which was raised up to 4 lakhs and repeat CT scan showed circumferential soft tissue thickening at the gastro-jejunostomy anastomosis site measuring ~30 × 16 mm in size with liver metastasis & lymphadenopathy. The patient deteriorated rapidly, and she died 5 months after surgery.{Figure 1}{Figure 2}{Figure 3}

Primary gastric yolk sac tumor is among the uncommon histologic variants of gastric tumor. The origin of yolk sac tumor in stomach is still unclear however few theories have been suggested, first, the germ cells appearing from the primitive streak, the splanchnopleure and the allantoic membrane of the embryo, migrate toward the gonadal ridge few of those germ cells remain at ectopic sites. Yolk sac tumor of the stomach tend to arise from these migrated germ cells.[5] Second theory as suggested in few reports states that the stomach develops from foregut with all the cells having complete genetic makeup, the tumor cells dedifferentiates into yolk sac phenotype.[8] Or may develop from embryonal rest[9] to best of our knowledge seven cases of pure gastric yolk sac tumor has been published in past [Table 1] with five cases having adenocarcinomatous component reported. The Pure Gastric YST have a median age of 65 years (36–62 years), only one case in pediatric age group is reported by Mandelia A et al.[6] Generally high levels of α-fetoprotein is detected in the serum of patients with gastric YST, other α-fetoprotein producing carcinoma of stomach includes hepatoid carcinoma, well-differentiated papillary or tubular type adenocarcinoma with clear cytoplasm and secondary tumors of gonadal origin needs to be ruled out before diagnosing this rare variant.[10] Histopathological feature of gastric yolk sac tumor are similar to the primary yolk sac tumor of gonads; however, large cell neuroendocrine carcinoma, undifferentiated carcinoma, mixed germ cell component, and adenocarcinoma component should also be looked for before diagnosing the pure yolk sac tumor following immunohistochemistry markers including Pan-CK, CK7, CK 20, CDX-2, synaptophysin, chromogranin, SALL-4 shall be designed as the first panel, positive expression for Pan CK and SALL-4 and negative expression of CK-7, CK20, CDX2, synaptophysin, and chromogranin would further rule out presence of adenocarcinoma component, undifferentiated carcinoma, and large cell neuroendocrine carcinoma, with SALL-4 expression further second panel may include CD30, PLAP, CD-117, OCT3/4, AFP, and beta HCG markers to rule out other germ cell component, hence all the differentials should be ruled out before diagnosing this rare subtype of gastric carcinoma.{Table 1}

The overall survival of gastric yolk sac tumor is one to seven months as published in previous case reports,[4] they present with poor prognosis and are usually diagnosed in advanced stage and usually presents with metastatic disease; however, one case was early diagnosed as it presented with polypoid mass, hence underwent gastrectomy with lymphadenectomy without recurrence.[3] This case is an addition to the AFP producing gastric carcinoma, which is a rare subtype and has poor prognosis with no standard therapeutic guidelines for treatment due to rarity of this subtype.

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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.

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