JOURNAL OF EVIDENCE BASED MEDICINE AND HEALTHCARE

Table of Contents

2018 Month : August Volume : 5 Issue : 32 Page : 2408-2410

PANCREATOBLASTOMA- A CASE REPORT

Mogili Vittal Reddy1, B. Swathi2, Kuchukulla Venkat Ram Reddy3, (Brig). R. Sathya Narayana Moorthy4, Gaddam Rama Krishna Reddy5

1. Postgraduate Student, Department of Radio-diagnosis, SVS Medical College and Hospital, Mahabubnagar, Telangana.
2. Postgraduate Student, Department of Radio-diagnosis, SVS Medical College and Hospital, Mahabubnagar, Telangana.
3. Professor and HOD, Department of Radio-diagnosis, SVS Medical College and Hospital, Mahabubnagar, Telangana.
4. Professor, Department of Radio-diagnosis, SVS Medical College and Hospital, Mahabubnagar, Telangana.
5. Professor, Department of Radio-diagnosis, SVS Medical College and Hospital, Mahabubnagar, Telangana.

Corresponding Author:
Dr. Mogili Vittal Reddy,
H. No. 1-49, Pakhala, Kodakandla,
Jangaon- 506222, Telangana.
E-mail: drvittalreddy@gmail.com
DOI: 10.18410/jebmh/2018/496

CASE PRESENTATION
A 3-year-old male child referred to the radiology department with complaints as explained by parents - loss of appetite & loss of body weight since 6 months, feeling of mass in upper abdomen since 1 month, and occasional vomiting since 1 month.

 

How to cite this article

Reddy MV, Swathi B, Reddy KVR, et al. Pancreatoblastoma- a case report. J. Evid. Based Med. Healthc. 2018; 5(32), 2408-2410. DOI: 10.18410/jebmh/2018/496

CASE PRESENTATION

A 3-year-old male child referred to the radiology department with complaints as explained by parents - loss of appetite & loss of body weight since 6 months, feeling of mass in upper abdomen since 1 month, and occasional vomiting since 1 month.

On local examination, a firm non-tender mass was palpable in epigastric region and left hypochondriac region.

 

DIFFERENTIAL DIAGNOSES

  • Neuroblastoma
  • Wilms Tumor
  • Hepatoblastoma and other primary liver tumours.
  • Non-Hodgkin’s Lymphoma-especially Burkitt’s lymphoma.

 

USG Findings-A large 10 x 8 cm well defined, predominantly hypoechoic soft tissue mass with few areas of anechogenicity noted in the left hypochondrium and epigastric region, probably arising from the pancreatic body and tail. The lesion was taking minimal flow on colour Doppler. The lesion was causing mass effect on the spleen, left kidney and stomach. Rest of the abdomen appeared normal.

Figure 1. USG Image Demonstrates a Large 10 x 8 cm Well Defined, Soft Tissue Density Mass with Cystic Areas arising from Body and Tail of Pancreas

CECT Abdomen Findings

On Plain CT- A large well defined heterogeneous soft tissue density mass with solid and cystic components noted arising from the body and tail region of the pancreas, there was no calcification noted.

 

On Post Contrast Imaging- The lesion showed moderate heterogeneous enhancement with non-enhancing necrotic areas. Neither vascular invasion nor hepatic metastasis noted.

 

Figure (2A, 2B, 2C). Plain CT Images Demonstrates a Large Well Defined, Heterogeneous Soft Tissue Density Mass with Solid and Cystic Components in the Body and Tail Region of Pancreas

 

 

Figure (3A, 3B, 3C). Contrast CT Demonstrates Moderate Heterogeneous Enhancement of the Mass Lesion with Multiple Non-Enhancing Necrotic Areas, without Invasion of any Adjacent Structures/Vascular Invasion

 

 

CLINICAL DIAGNOSIS

Pancreatoblastoma.

 

DISCUSSION OF MANAGEMENT

The patient was taken for surgery by the paediatric surgery department.

Figure 4. Intra Operative Image Shows a Large Lobulated, Well Capsulated Lesion Noted in Body and Tail of Pancreas

 

Intra Operative Findings

A large well defined lobulated well circumscribed lesion with well-defined capsule noted in body and tail of pancreas. Which was resected and sent for histopathological examination.

 

PATHOLOGICAL DISCUSSION

Histopathological Findings

Encapsulated tumor composed of cellular stroma separating tumor into nests and trabeculae. The cells form solid structures resembling acini and short tubules. Cells lining the acini are small to medium sized round to polygonal with scanty eosinophilic cytoplasm. Eosinophilic secretions are noted within the lumen of some of the acini, focal capsular invasion and sub capsular vascular tumor emboli are also noted and diagnosis of pancreatoblastoma was made.

       Paediatric Pancreatic tumours are quite rare in children, causing less than 0.2% of malignant paediatric deaths.1 They are usually classified as exocrine or endocrine tumors. The most common exocrine pancreatic tumours in paediatric population are pancreatoblastoma and adenocarcinoma. Pancreatoblastoma arises from pancreatic acinar cells, these cells represent persistence of foetal anlage of the acinar cells.

       Pancreatoblastoma also known as” infantile carcinoma of pancreas”, is the most common pancreatic neoplasm of childhood.2 This rare epithelial tumor, which is often misdiagnosed as neuroblastoma or hepatoblastoma,3 has a 2:1 male to female ratio.4,2 The mean age at diagnosis is 4 years, but it can manifest at any time from the newborn period to adulthood.4 More than half of reported cases are in Asians.5,6 Congenital cases of pancreatoblastoma have been described in association with Beckwith- Wiedemann syndrome, and these are predominantly cystic in nature.7,8

Patients usually present with abdominal distension or a large, palpable abdominal mass. The mass may be associated with nonspecific symptoms, including failure to thrive, epigastric pain, anorexia, vomiting, diarrhoea and weight loss.2 Obstructive jaundice may also be present. The serum α-fetoprotein level is elevated in 25% to 50% of patients, and the tumor may secrete adrenocorticotropic hormone.4,5 Although usually located in the pancreatic head,9 pancreatoblastoma can be located anywhere in the pancreas; it may be exophytic or may entirely replace the pancreas.2 It tends to be a large mass with a diameter of upto 7 to 18 cm at the time of presentation. It is usually solitary, well defined, and surrounded by a fibrous capsule.9,2 Metastasis are most commonly seen in liver but may also occur in regional lymph nodes, the lungs and rarely in bone.5 Local invasion of the bowel and peritoneal cavity can also occur.2 Metastatic disease is rare at the time of presentation. When metastatic disease is present the prognosis is typically poor.4

In general imaging demonstrates a solid mass with appearance suggestive of but not specific for pancreatoblastoma.10 Sonography demonstrates a well –demarcated, predominantly hypoechoic mass,5 which may have central areas of low echogenicity.2

CT demonstrates a focal mass with attenuation generally lower than that of liver. The mass may contain areas of low attenuation, and there is mild, heterogeneous contrast enhancement.5 The pancreatoblastoma usually is large and well defined, and it may be lobulated.3 Metastases to the Liver tend to be hypodense and may contain areas of central necrosis.5 Vascular encasement of the mesenteric vessels and inferior vena cava may develop, and calcifications can be present, which may make differentiation from neuroblastoma difficult.5

MR features of pancreatoblastoma are suggestive but not specific, with variable signal intensity on T1- weighted images and high signal intensity on T2-weighted images. The mass usually demonstrates enhancement after contrast administration.4,2 MR and CT can show direct invasion of adjacent structures, including the spleen, left kidney, left adrenal gland and omentum.5 Given the nonspecific imaging findings of pancreatoblastoma, the diagnosis is generally established by percutaneous biopsy.10

Treatment consists of surgical excision, with chemotherapy administered for metastatic disease.2 Radiation therapy is used for local recurrence or incomplete resection.10,11 Although some patients are cured with excision alone, recurrence has been described in upto 60% of patients.11

 

FINAL DIAGNOSIS

Pancreatoblastoma.

 

REFERENCES

  1. Tsukimoto I, Watanabe K, Lin JB, et al. Pancreatic carcinoma in children in Japan. Cancer 1973;31(5):1203-1207.
  2. Johnson PR, Spitz L. Cysts and tumors of the pancreas. Semin Pediatr Surg 2000;9(4):209-215.
  3. Vaughn DD, Jabra AA, Fishman EK. Pancreatic disease in children and young adults: evaluation with CT. Radio Graphics 1998;18(5):1171-1187.
  4. Nijs E, Callahan MJ, Taylor GA. Disorders of the pediatric pancreas: imaging features. Pediatr Radiol 2004;35(4):358-373.
  5. Roebuck DJ, Yuen MK, Wong YC, et al. Imaging features of pancreatoblastoma. Pediatr Radiol 2001;31(7):501-506.
  6. Solcia E, Capella C, Kloppel G. Atlas of tumor pathology: tumors of the pancreas. Washington, DC: Armed Forces Institute of Pathology 1997.
  7. Pelizzo G, Conoscenti G, Kalache KD, et al. Antenatal manifestation of congenital pancreatoblastoma in a fetus with Beckwith-Wiedemann syndrome. Prenat Diagn 2003;23(4):292-294.
  8. Drut R, Jones MC. Congenital pancreatoblastoma in Beckwith-Wiedemann syndrome: an emerging association. Pediatr Pathol 1988;8(3):331-339.
  9. Herman TE, Siegel MJ. CT of the pancreas in children. AJR 1991;157:375-379.
  10. Enriquez G, Vazquez E, Aso C, et al. Paediatric pancreas: an overview. Eur Radiol 1998;8(7):1236-1244.
  11. Shorter NA, Glick RD, Klimstra DS, et al. Malignant pancreatic tumors in childhood and adolescence: the memorial Sloan-Kettering experience, 1967 to present. J Pediatr Surg 2002;37(6):887-892.