Sir,We read with interest the article entitled, “Pancreatoblastoma masquerading as hepatoblastoma: A diagnostic dilemma.”[1] The authors have reported a very challenging clinicoradiologic diagnostic problem. We report a similar case wherein a 7-month-old male infant with abdominal lump, low-grade fever, and slightly elevated serum alpha-fetoprotein (AFP >2000 ng/ml) was initially suspected to be a retroperitoneal germ cell tumor (GCT); however, review of radiology and histopathology revealed the tumor to be a pancreatoblastoma.Ultrasound examination revealed a large mixed echogenic mass arising from the retroperitoneum and indistinguishable from pancreatic body and tail. The characteristic radiologic findings on computed tomography (CT) were a well-defined, heterogeneously enhancing retroperitoneal tumor with tumor thrombus in the splenoportal venous axis and multiple venous collaterals [Figure 1].
Figure 1
Contrast-enhanced computed tomography showing a well-defined heterogeneously enhancing retroperitoneal mass with tumor thrombus at porta hepatis
Contrast-enhanced computed tomography showing a well-defined heterogeneously enhancing retroperitoneal mass with tumor thrombus at porta hepatisFine-needle aspiration cytology showed malignant cells of undifferentiated origin, possibly – sarcoma or carcinoma. Awaiting histopathological conformation by incisional biopsy, an initial diagnosis of a large retroperitoneal GCT was made and neoadjuvant chemotherapy initiated. There was no clinical response to chemotherapy and the patient developed gross ascites with anasarca. A review of the CT findings of thrombosis along the splenoportal axis with search of literature prompted a rethink of the diagnosis, keeping pancreatoblastoma as a more likely possibility. Characteristic immune histochemistry (positive for cytokeratin [CK], vimentin, CK 7, and AFP) confirmed the diagnosis of pancreatoblastoma. The patient responded well to cisplatin- and doxorubicin-based chemotherapy (PLADO) and a spleen-preserving distal pancreatectomy was performed after three cycles of neoadjuvant chemotherapy. Postoperatively, two more cycles of chemotherapy were given and the patient was followed up with serial AFP levels and ultrasound examination.Our case highlights the fact that giving undue importance to the raised serum AFP levels may lead to the misdiagnosis. Drozynska et al.[2] also reported a case of pancreatoblastoma misdiagnosed as a GCT on the basis of the clinical and radiologic signs with raised serum AFP levels and emphasized the importance of tissue biopsy in making the correct diagnosis. In advanced pancreatic tumors, including cases of pediatric pancreatoblastoma, tumor invasion and thrombosis of the portal venous system have been reported and are characteristic findings which point to the diagnosis.[34] In retrospect, the presence of thrombosis in the splenoportal venous axis along with the raised serum AFP levels and the CT findings of an upper abdominal, retroperitoneal mass should have avoided the initial misdiagnosis in our patient and pancreatoblastoma should be in differential diagnosis.
Figure 1