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An official publication of the Middle-Eastern Association for Cancer Research
Clinical Cancer Investigation Journal
ARTICLE
Year: 2022   |   Volume: 11   |   Issue: 6   |   Page: 21-24     View issue

Frantz's Tumor an Unusual Pancreatic Neoplasm with Rare Presentation

 

Banyameen Iqbal1*, Tushar Kambale1, Charusheela Gore1, Vidya Vishwanathan1, Arpana Dharwadkar1

1Department of Pathology, D.Y Patil Medical College, Hospital and Research Centre, Dr. D.Y Patil Vidyapeeth, Pimpri, Pune – 411018, India.


Abstract

A solid pseudopapillary tumor of the pancreas (SPT) is alternatively called a 'Frantz tumor.' It was first named after its discoverer in the year 1959. This tumor has been previously designated as papillary epithelial neoplasm, low-grade papillary neoplasm, and solid and papillary neoplasm of the pancreas. It is a rare pancreatic neoplasm. It typically affects young people and has a female predilection. Its histogenesis is rather debatable. Acinar, endocrine, ductal, and progenitor cells have been postulated as a possible starting point of this tumor. It has a relatively favorable prognosis with low malignant potential. It accounts for less than 3% of all exocrine pancreatic neoplasms. Although most SPTs behave as benign tumors, up to 15% of the cases can show malignancy. We are presenting a rare case of a 19-year-old female who came with epigastric abdominal pain. Surgical excision was done, and a histopathology/Immunohistochemistry examination confirmed it as a Frantz tumor.

Keywords: Frantz tumour, Pancreas, Papillary lesions, Solid lesions, Pseudo-papillary


Introduction

SPT is a very rare neoplasm of the pancreas. It typically affects young women in their third decade of life and has a relatively better prognosis. It has a low malignant potential. It is also known as the 'Frantz tumor,' described first by Dr. Frantz in the 1950s and later known as a "papillary tumor of the pancreas, benign or malignant." Its histogenesis is rather debatable. Acinar, endocrine, ductal, and progenitor cells have been postulated as a possible starting point of this tumor. It is a well-encapsulated tumor and accounts for less than 3% of all exocrine pancreatic neoplasms.[1, 2] It is a well-encapsulated mass, and almost 50 to 60% of the cases arise in the body or tail region of the pancreas.[3] It is usually asymptomatic or minimally symptomatic. Although most SPTs behave as benign tumors, up to 15% of the cases can show malignancy and manifest as metastases or vascular and perineural invasion.[4]

Case report

A 19-year-old female came with epigastric pain for the last 6 months duration. She had no previous history of hospitalization. Her vitals were within normal limits. A physical examination was done, which showed mild tenderness in the epigastric region. There were no palpable lesions, and bowel sounds were normal. She gave no history of vomiting/jaundice/loose stools/abdominal distension/trauma/fever/weight loss/loss of appetite/Diabetes/ hypertension/ tuberculosis/asthma. Routine laboratory (biochemical and hematological) tests were in the normal range. Abdominal Ultrasonography revealed a solid, round to-oval isoechoic lesion in the vicinity of the head of the pancreas. Computed Tomography (CT) scan abdomen revealed a well-defined heterogeneous lesion of size 31 x 29 x 26mm in the head of the pancreas. (Figure 1a) MR Imaging revealed (Axial T1WI, Axial T2WI) a well-defined, exophytic lesion with a regular smooth margin of similar size in the head of the pancreas, appearing hypo intense on T1WI and hyper-intense on T2WI. Imaging suggested a tumor with a mostly solid component of the head of the pancreas (Figure 1b).

 

 

a)

b)

Figure 1. a) The image shows a well-defined heterogeneous lesion of size 31 x 29 x 26mm in the head of the pancreas. b) The image shows a well-defined, exophytic lesion with regular smooth margins in the head of the pancreas, appearing hypo intense on T1WI and hyper-intense on T2WI.

Endoscopic ultrasound-guided FNA revealed cellular smears showing multiple layers of monomorphic cuboidal tumor cells arranged in papillary fronds with central thin fibrovascular stroma. The tumor cells have granular eosinophilic cytoplasm, small round nuclei, fine chromatin, and occasional nuclear grooves. No significant mitotic activity (Figure 2a).

The frozen section of enucleated tumor of the pancreas showed a neoplasm comprising small uniform tumor cells in multiple layers arranged around hyalinized blood vessels appearing as pseudo papillae (Figure 2b).

a)