Metastatic adenoid cystic carcinoma – An unusual neoplastic lesion of the liver

Ashish Tapadia; Karattupalayam Sampath Mouleeswaran; Jacob Nikhil Thomas; Joy Varghese

doi:10.4103/ghep.ghep_12_20

CASE REPORT

Year : 2021 | Volume : 1 | Issue : 1 | Page : 40-42

Metastatic adenoid cystic carcinoma – An unusual neoplastic lesion of the liver

Ashish Tapadia¹, Karattupalayam Sampath Mouleeswaran², Jacob Nikhil Thomas¹, Joy Varghese¹
¹ Department of Hepatology and Transplant Hepatology, Gleneagles Global Health City, Chennai, Tamil Nadu, India
² Department of Pathology, Gleneagles Global Health City, Chennai, Tamil Nadu, India

Date of Submission	14-Aug-2020
Date of Decision	15-Aug-2020
Date of Acceptance	17-Aug-2020
Date of Web Publication	04-Dec-2020

Correspondence Address:
Jacob Nikhil Thomas
Department of Hepatology and Transplant Hepatology, Gleneagles Global Health City, Chennai - 600 100, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ghep.ghep_12_20

Abstract

Adenoid cystic carcinoma (ACC) is an uncommon tumor of the salivary glands and is known for its delayed presentation of distant metastasis. However, delayed presentation of metastatic ACC in the liver without primary recurrence is rare, and literature is limited. In this case report, we present a case of metastatic ACC involving the liver without primary recurrence 6 years after radical excision of submandibular ACC.

Keywords: Adenoid cystic carcinoma, liver tumor, metastasis

How to cite this article:
Tapadia A, Mouleeswaran KS, Thomas JN, Varghese J. Metastatic adenoid cystic carcinoma – An unusual neoplastic lesion of the liver. Gastroenterol Hepatol Endosc Pract 2021;1:40-2

How to cite this URL:
Tapadia A, Mouleeswaran KS, Thomas JN, Varghese J. Metastatic adenoid cystic carcinoma – An unusual neoplastic lesion of the liver. Gastroenterol Hepatol Endosc Pract [serial online] 2021 [cited 2021 Jan 16];1:40-2. Available from: http://www.ghepjournal.com/text.asp?2021/1/1/40/302214

Introduction

Adenoid cystic carcinoma (ACC) is an uncommon salivary gland tumour and it’s propensity for delayed presentation of distant metastasis. However, delayed presentation of metastatic ACC in the liver without primary recurrence is rare and literature is limited. There are only a few case reports regarding recurrence in the liver as bone and lung metastasis are common. In this case report, we present a case of metastatic ACC involving the liver in a female patient who had undergone right submandibular excision for submandibular ACC.

Case Report

A 42-year-old female from Saudi Arabia presented with the complaints of vague abdominal discomfort for the past 2 years with a progressively increasing mass over the right side of the abdomen. She had lost approximately 10 kg in her weight. There was no history of jaundice, fever, vomiting, or gastrointestinal bleed. Six years ago, she had a swelling over the right submandibular region and was diagnosed with submandibular gland adenoid cystic carcinoma (ACC). Her positron emission tomography (PET) scan showed no evidence of distant metastasis. Hence, she underwent radical excision of the involved gland, followed by adjuvant radiotherapy.

At present, there was a nodular mass felt over the right half of her abdomen, which was nontender, hard in consistency and was extending up to the right iliac fossa. Her baseline investigations revealed hemoglobin: 13.8 g/deciliter, white cell count: 6010 cells/mm cube, platelet count: 212,000 cells/mm cube, total bilirubin: 1.05 mg/dl, alanine aminotransferase: 131 IU, aspartate aminotransferase: 67 IU, alkaline phosphatase: 362, gamma glutamyltransferase: 422, total protein: 6.5 g/dl, serum albumin: 3.8 g/dl, serum creatinine: 0.5 mg/dl, and alpha-fetoprotein: 3.5 units. Serological tests for hepatitis B, hepatitis C, and HIV infection were negative.

Contrast-enhanced computed tomography scan revealed multiple heterogeneously enhancing lesions throughout the liver. The large one was an exophytic growth from the right lobe of the liver measuring 15.7 cm × 19 cm × 15 cm in size [Figure 1] and [Figure 2]. Moreover, PET-scan revealed multiple focal lytic lesions over iliac bones, vertebrae, and left 7^th rib.

Figure 1: Computed tomography scan with intravenous contrast showing the multiple liver lesions described

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Figure 2: Coronal view shows the extent of the tumor up to the right iliac fossa

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Biopsy from the liver lesion showed areas of cribriform pattern (characteristic feature of ACC) comprised pseudocyst like spaces that that resemble that of “Swiss Cheese” [Figure 3] and [Figure 4]. On hematoxylin and eosin staining, Immunohistochemical staining (IHC) showed luminal cytoplasmic positivity for CD117 [Figure 5], cytokeratin 7 [Figure 6] and myoepithelial cells displayed nuclear positivity for P63 [Figure 7]. All other IHC markers including Hep Par 1, CA 19-9, chromogranin, synaptophysin, and CDX 2 were found to be negative. Hence, the diagnosis of metastatic ACC was made.

Figure 3: Cribriform pattern of adenoid cystic carcinoma demonstrated by the black arrow

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Figure 4: Similar findings being demonstrated at a lower power

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Figure 5: Cytoplasmic positivity for CD117 seen under × 400

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Figure 6: Cytokeratin-7 expression of the luminal cells seen under × 200

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Figure 7: P63 - nuclear positivity in the myoepithelial cells of cribriform glands (×400)

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Discussion

ACC is a relatively rare tumor, accounting for approximately 5%–10% of all salivary gland tumors and more commonly arise from the submandibular gland and other minor salivary glands (>40%).^[1] ACC is known for its prolonged clinical course with a high rate of distant metastases. The usual sites of metastases are the lungs and bone, whereas brain and liver are rarely involved.^[2],[3] The reported rates of liver metastasis in ACC range from 2% to 20% and it is usually a disseminated disease. On the other hand, there are few cases reports shows that liver metastasis might be an initial presentation of ACC.^[4‐6] Distant metastasis usually manifest clinically after a long period even many years after of primary diagnosis and treatment.^[7],[8] Median survival in such metastatic disease would be (approximately 3–4 years) better than other solid organ tumors.^[7] In the era of immunohistochemistry (IHC), IHC markers help in differentiating metastatic ACC from other neoplastic lesions of the liver.^[6],[9] Management for metastatic ACC would be palliative chemotherapy.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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