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| Year : 2009 | Volume
: 52
| Issue : 4 | Page : 537-539 |
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| Coexistent gastric primary choriocarcinoma and adenocarcinoma |
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Muktha R Pai1, Alka M Mathai1, Suneet Kumar1, Shivananda Prabhu2
1 Department of Pathology, Kasturba Medical College, Mangalore, Karnataka, India
2 Department of Surgery, Kasturba Medical College, Mangalore, Karnataka, India
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| Date of Web Publication | 1-Oct-2009 |
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 Abstract | | |
Choriocarcinomas are commonly gestational and intrauterine or gonadal in origin. Nongestational and extragonadal disease, especially in the stomach is extremely rare with a poorer prognosis and early metastasis. We herein describe a case of primary gastric choriocarcinoma with liver metastasis in a 69-year-old male. The patient presented with abdominal pain and irregular bowel habits of one month duration. Both endoscopic and radiographic imaging revealed an ulcerated and necrotic circumferential tumor in the pyloric antrum of the stomach. The histopathologic findings of the resected tumor were that of an extensively necrotic and hemorrhagic neoplasm with combined choriocarcinomatous and adenocarcinomatous element. A strongly positive immunohistochemical staining for β- subunit of human chorionic gonadotropin (β-HCG) in the choriocarcinomatous component and a proper correlation with elevated serum β-HCG levels confirmed the diagnosis. Keywords: Adenocarcinoma, choriocarcinoma, gastric tumor, β-human chorionic gonadotropin, liver metastasis
How to cite this article:
Pai MR, Mathai AM, Kumar S, Prabhu S. Coexistent gastric primary choriocarcinoma and adenocarcinoma. Indian J Pathol Microbiol 2009;52:537-9 |
| Introduction | |  |
Primary gastric choriocarcinomas (PGCs) are rare, accounting for 0.08% of gastric cancers. [1] Although highly malignant, they are often undetected preoperatively. Critical evaluation and identification of the rare histological patterns of gastric cancer play a significant role in the diagnosis and determination of clinical outcome of the patient. We herein describe a rare case of combined PGC and adenocarcinoma in an elderly male. The typical histological pattern, immunohistochemistry and a proper correlation with serum β-human chorionic gonadotropin (β-HCG) levels enabled a correct diagnosis.
| Case Report | | |
A 69-year-old male presented with diffuse and intermittent abdominal pain and irregular bowel habits of one month duration. He had malena and also complained of loss of appetite and loss of weight of more than seven kilograms in the past one month. He was on treatment for hypertrophic cardiomyopathy and had vesical calculi and benign prostatic hyperplasia in the past which was treated. On examination, the patient was pale and had bilateral pitting pedal edema. The laboratory investigations showed low hemoglobin of 6.5 mg/dl. Features of hypertrophic cardiomyopathy were evident on echocardiography. On ultrasonogram and computed tomography scan, the stomach wall was 5-mm thick at the pyloric region narrowing the lumen and extended to the first part of the duodenum. A suspicious liver nodule was also present. Gastric endoscopy revealed a large ulcerated tumor in the pyloric antrum. A biopsy was taken and interpreted as adenocarcinoma. The patient underwent subtotal Billroth II gastrectomy with gastrojejunostomy. Intraoperatively, the tumor was adherent to the liver, transverse mesocolon and pancreas.
Grossly, the resected specimen consisted of stomach measuring 14 cm Χ 4.5 cm Χ 3.5 cm with 3 cm of duodenum and greater omentum. The gastric mucosa was ulcerated with a large necrotic and hemorrhagic tumor of size 5 cm in diameter on the lesser curvature involving the posterior wall of the pyloric region. The tumor had elevated margins and infiltrated the entire thickness of the wall up to the serosa forming hemorrhagic fungating nodules [Figure 1]. The rest of the stomach showed thickened and edematous mucosal rugae.
On histology, the tumor had dual components closely adjoining each other at places [Figure 2]. One component comprised neoplastic columnar cells arranged in tubules and papillae infiltrating the mucosa and submucosa exhibiting a well-differentiated adenocarcinomatous pattern in the ulcerated area. Diastase-resistant periodic acid-Schiff stain and mucicarmine stain highlighted the intracytoplasmic mucin in these tumor cells. The other component was present in the hemorrhagic nodular part and showed extensive areas of necrosis rimmed predominantly by large polyhedral cytotrophoblastic tumor cells (CTCs) with abundant foamy eosinophilic cytoplasm, distinct cell borders and large vesicular nuclei having prominent macronucleoli, capped by streaming masses of multinucleated syncytiotrophoblastic tumor cells (STCs) at intervals; the pattern was that of a choriocarcinoma accounting for approximately 70% of the tumor infiltrating the muscularis externa and serosa. The intervening areas showed an undifferentiated carcinoma. Both resected margins of the specimen were free of tumor. No regional lymph node metastasis or omental seeding was identified.
On immunohistochemistry, the tumor cells showed strong diffuse immunopositivity with pan-cytokeratin AE1 and AE3. The sheets of trophoblastic tumor cells, especially STCs showed strong immunopositive staining for β-HCG [Figure 3]. The adenocarcinomatous element at the peripheral infiltrating margins and the poorly differentiated areas showed a weakly positive reactivity against β-HCG antibody. However, the normal gastric mucosa did not reveal any HCG-producing cells. In view of the histology, subsequent serum β-HCG levels showed marked elevation (2179 mIU/mL). Based on the biochemical, histopathological and immunohistochemical features, a diagnosis of PGC coexistent with a moderately differentiated tubulo-papillary adenocarcinoma was made.
Postoperatively, the patient was stable. Six weeks later, the repeat laboratory tests showed markedly elevated serum β-HCG levels of 30,496 mIU/mL and serum carcinoembryonic antigen levels of 137.7 ng/ml. Radiological imaging showed a nodular lesion in the liver consistent with metastasis. He received only conservative treatment because of his poor general health and was advised regular follow-up on discharge.
| Discussion | | |
PGC is a rare, rapidly growing and widely metastasizing β-HCG-producing epithelial tumor arising from the trophoblastic germ cells; choriocarcinomas metastasizing to the stomach are relatively common. [2],[3] Apart from gestational choriocarcinomas, they occur as primary neoplasm in the extragonadal sites such as mediastinum, lung, liver, breast, prostate, urinary bladder, stomach and nose; stomach being an extremely rare site. [2],[3] They occur either in pure form, accompany adenocarcinoma or occur in association with nontrophoblastic gonadal tissue. [4] A metastasis from a primary trophoblastic tumor, particularly in the gonads and uterus in females has to be ruled out. [5]
The various theories regarding pathogenesis include its development from a gastric teratoma or a displaced gonadal anlage or a metastasis from an intrauterine lesion. However, based on its occurrence with adenocarcinoma and common factors regarding clinical features, age, sex and site distribution, the widely accepted retrodifferentiation theory describes the dedifferentiation of the adenocarcinoma cells to embryonal ectoderm with subsequent trophoblastic precursor cell metaplasia; choriocarcinoma overgrows and eliminates the original adenocarcinoma. [3],[4] Pure choriocarcinoma arises de novo from the HCG-producing cells of the normal gastric mucosa.
The distribution pattern of HCG-producing cells within the tumor noted in the present case correlated with that of Yakeishi et al. [6] In contrast, the normal gastric mucosa did not show any HCG-producing cells in the present case and diffuse positivity for β-HCG was shown by 70-80% of the cytotrophoblasts as noted by Ramponi et al.[7]
Choriocarcinomas are often undiagnosed preoperatively because of their association with adenocarcinoma or other elements such as neuroendocrine cell carcinoma, yolk sac or hepatoid carcinoma. [8],[9] The endoscopic biopsy taken randomly may be inadequate to characterize the precise histological pattern, like in the present case. Larger and multiple biopsies from the whole tumor are recommended for proper diagnosis when extensive hemorrhage and necrosis are present so that no minor foci of other pathological components are missed. [10]
Elevated serum β-HCG levels or presence of β-HCG-producing tumor cells per se has no correlation with tumor stage, grade or prognosis. [4],[10] Choriocarcinomas have rapid hematogenous dissemination unlike the lymphatic spread of adenocarcinomas. The prognosis is poor with very high mortality and a survival period of less than one year. Chemotherapy regimes are not effective as compared with the gestational choriocarcinomas. However, curative resection, appropriate chemotherapy and absence of synchronous liver metastasis are favorable prognostic indicators. [1]
In our patient, the intervening undifferentiated areas representing gradual transition of adenocarcinoma to choriocarcinoma and the distribution of HCG-producing cells within the adenocarinomatous and undifferentiated component favored the origin of choriocarcinoma from a preexisting adenocarcinoma. Absence of a germ cell tumor elsewhere excluded the possibility of a metastasis. Since the tumor was adherent to the liver, colon and pancreas, it was difficult to predict whether the resection was complete or not. The presence of synchronous liver metastasis, doubtful residual tumor and absence of chemotherapy suggested a poorer prognosis as evidenced by a new elevation of serum β-HCG levels in the follow-up period. Due to the fewer cases reported and the scant data available regarding a long-term follow-up, further studies are required to determine the potential influence of surgery and chemotherapy on the clinical course of these kind of disseminating neoplasms.
| References | | |
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| 2. | Noguchi T, Takeno S, Sato T, Takahashi Y, Uchida Y, Yokoyama S. A patient with primary gastric choriocarcinoma who received a correct preoperative diagnosis and achieved prolonged survival. Gastric Cancer 2002;5:112-7. |
| 3. | Liu AY, Chan WY, Ng EK, Zhang X, Li BC, Chow JH, et al. Gastric choriocarcinoma shows characteristics of adenocarcinoma and gestational choriocarcinoma: a comparative genomic hybridization and fluorescent in situ hybridization study. Diagn Mol Pathol 2001;10:161-5. |
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| 6. | Yakeishi Y, Mori M, Enjoji M. Distribution of beta - human chorionic gonadotropin-positive cells in noncancerous gastric mucosa and in malignant gastric tumors. Cancer 1990;66:695-701. |
| 7. | Ramponi A, Angeli G, Arceci F, Pozzouli R. Gastric choriocarcinoma: An immunohistochemical study. Pathol Res Pract 1986;181:390-6. |
| 8. | Lauwers GY. Epithelial neoplasms of the stomach. In: Odze RD, Goldblum JR, Crawford JM, editors. Surgical pathology of the GI Tract, Liver, Biliary Tract and Pancreas. Philadelphia: Saunders; 2004. p. 409-27. |
| 9. | Rosai J, editor. Rosai and Ackerman's surgical pathology. 9th ed. St. Louis: Mosby; 2004. |
| 10. | Hirano Y, Hara T, Nozawa H, Oyama K, Ohta N, Omura K, et al. Combined choriocarcinoma, neuroendocrine cell carcinoma and tubular adenocarcinoma in the stomach. World J Gastroenterol 2008;14:3269-72. |
Correspondence Address:
Alka M Mathai
Department of Pathology, Kasturba Medical College, P.O. Box – 53, Mangalore - 575 001, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.56155
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