Mucinous carcinoma of gallbladder in a young female: A rare presentation

Mucinous carcinoma of gallbladder in a young female: A rare presentation: Nidhi Awasthi, Abhishant Pandey



International Journal of Health & Allied Sciences 2020 9(1):78-79



Mucinous carcinoma of the gallbladder is an uncommon variant encountered predominantly in the elderly population. An unusual occurrence of this relatively uncommon malignancy is described herewith in a young female.

Introduction

Gallbladder (GB) is the most common site for malignancy within the biliary tract, and it is the fifth most common site among gastrointestinal tract-related organs. A female predominance is seen with male-to-female ratio being approximately 1:3. Most of the patients are above 50 years of age, with a mean age of 65 years.^[1] Approximately 90% of all GB malignancies are adenocarcinomas; however, other variants have also been described. Considering the rarity of malignancies other than adenocarcinomas in GB and that too in a young patient, we herewith describe a case report of occurrence of mucinous carcinoma in a young female.

Case Report

A 30-year-old female presented with the complaint of right upper abdominal pain. On clinical examination and ultrasound investigation, a provisional diagnosis of chronic cholecystitis was made, following which an open cholecystectomy was done. The specimen was received in SRL Lucknow Laboratory for histopathology. Macroscopic examination [Figure 1]a showed an opened up GB specimen with a papillary growth at the fundus, measuring approximately 2 cm × 1.5 cm. Cut surface of the growth had a grayish-white gelatinous appearance. Mucosa in rest of the areas appeared gelatinous and flattened. Wall thickness varied from 0.2 to 2 cm. Single lymph node was identified embedded in the wall, near the neck measuring 1 cm in maximum dimension. Single stone was found impacted at the neck. Representative sections were taken and subjected to routine processing with staining by hematoxylin and eosin. Microscopic examination showed a malignant epithelial neoplasm composed of well-formed glands in the area of papillary growth lined by atypical cuboidal to columnar cells having pleomorphic, vesicular-to-hyperchromatic nuclei with abnormal coarse chromatin and distinct-to-prominent nucleoli at places. Nuclear crowding and stratification were evident in focal areas with loss of polarity. Intraluminal necrotic debris was seen in fair number of glands along with few mitosis. Underlying layers displayed large pools of extracellular mucin, constituting 60%–70% of tumor volume, containing clusters of tumor cells admixed with signet ring type cells [Figure 1]d. Tumor cells were seen penetrating the muscularis propria and reaching up to the serosa at places. Angiolymphatic and perineural invasion was present. Both the neck resection margin and lymph node were found positive for tumor. Additional periodic acid–Schiff (PAS) stain [Figure 1]b and [Figure 1]c was done for demonstration of extracellular mucin and signet ring cells. Considering the characteristic findings on histomorphology and PAS stain, a diagnosis of mucinous carcinoma was rendered.

Figure 1: (a) Gross picture showing papillary growth at fundus with rest of the mucosa showing gelatinous areas. (b) Papillary tumor with well-formed glands and underlying mucin pools (PAS, ×100). (c) Signet ring cells and pools of mucin (PAS, ×400). (d) Signet ring cells in pools of mucin (H and E, ×400) Click here to view

Discussion

Mucinous carcinoma is an uncommon variant of GB cancer. It accounted for 2.5% of all GB carcinomas in a reported series by Dursun et al.^[2] Few case reports of the entity have been found in literature,^{[3],[4],[5],[6],[7],[8],[9]} describing its occurrence in an age range from 48 to 73 years. Right upper abdominal pain is the most common presenting symptom, which may be associated with nausea, vomiting, anorexia, jaundice, and weight loss. Magnetic resonance cholangiopancreatography has been described to be beneficial in suspecting a preoperative diagnosis of mucinous carcinoma of GB.^[7],[8],[9] Histologically, carcinomas with stromal mucin deposition are categorized into pure mucinous (colloid) and mucinous carcinomas.^[1] Pure mucinous or colloid carcinomas have 90% or more of extracellular mucin, which is exceedingly rare.^[10] In mucinous carcinomas, extracellular mucin constitutes more than 50% but <90% of the tumor volume.^[1] These tumors are typically large and more advanced at the time of diagnosis and exhibit more aggressive behavior than do ordinary GB carcinomas.^[2] Immunophenotypically, they differ from conventional GB adenocarcinomas by MUC2 positivity, from intestinal carcinomas by an often inverse CK7/20 profile, from pancreatic mucinous carcinomas by CDX2 negativity, and from mammary colloid carcinomas by a lack of MUC6.^[2] To summarize, although mucinous carcinoma of GB is an uncommon aggressive tumor usually encountered in the elderly population, it can rarely present in younger individuals too as described in this case report.

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Conflicts of interest

There are no conflicts of interest.



 

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