Ahmad Abulawi, MBBS1, Hala Abdelwahab, MD2, Omar Tageldin, MD2, Asra Batool, MD2 1Albany Medical College, Albany, NY; 2Albany Medical Center, Albany, NY
Introduction: Small cell carcinoma (SCC) cases typically occur in the lungs, accounting for 25% of lung carcinomas. Extrapulmonary small cell carcinoma (EPSCC) is rare, accounting for 2-4% of SCC cases. The liver is one of the organs to develop primary SCC with only a few reported cases in the literature. We report a case of a patient with primary SCC of the liver presenting with failure to thrive.
Case Description/Methods: A 65-year-old male former smoker, presented with fatigue, weight loss (30 pounds over 5 months), and anorexia. Physical exam was significant for cachexia, mild abdominal distention, and right upper quadrant tenderness. Laboratory studies were significant for AST, of 59 U/L, ALT 71 U/L, ALP 514 U/L; GGT 200 U/L; and total bilirubin, 1.8 mg/dL. The Hepatitis panel was negative. CA19-9, alpha-fetoprotein, and CEA were not elevated. Chest/Abdomen/Pelvis computed tomography (CT) with contrast was remarkable for heterogeneous enhancement mass measuring 7.2 x 6.8 x 5 cm in the liver. Positron Emission Tomography (PET) scan revealed a 7 x 6.2 cm mass lesion in the liver with metabolic active disease at the periphery and central necrosis. No other hypermetabolic lesions in the body were seen. Fine needle aspiration cytology (FNAC) and immunohistochemistry showed features of primary small cell carcinoma of the liver. The tumor cells show positive staining for Chromogranin, CD56, and CK8/18. The tumor cells are negative for CK7, CK20, Synaptophysin, PIN-4, PAS, and CA19-9. The tumor was inoperable, therefore palliative chemotherapy was considered the appropriate option. However, the patient and family opted for comfort care and the patient was discharged to home hospice.
Discussion: EPSCC has been recognized as a distinct clinical entity. A rare tumor that represents 0.1-0.4 % of all cancers. Most SCC cases occur in the lungs and bronchial tree accounting for small cell lung carcinoma. However, EPSCC is becoming more common and cases have been reported in other organs. SCC of the liver as in our patient is extremely rare. To differentiate between EPSCC and metastatic pulmonary small cell carcinoma, the patient should have a normal CT of the chest, negative bronchoscopy or PET scan, and unremarkable sputum cytology. Diagnosis requires tissue biopsy and immunohistochemistry. There is no established standard treatment for primary SCC of the liver, but chemotherapy and surgical resection were tried with good response. Unfortunately, our patient refused chemotherapy because of his poor health condition.
Figure: Figure 1.A. H&E-stained section showing a hyperchromatic small cell with nuclear molding and crush artifact infiltrating the liver. Figure 1.B. The tumor cells show positive staining for Chromogranin. The tumor cells were also positive for CD56 and CK8/18 and negative for CK7, CK20, Synaptophysin, PIN-4, PAS, and CA19-9 stains (Not shown).
Ahmad Abulawi indicated no relevant financial relationships.
Hala Abdelwahab indicated no relevant financial relationships.
Omar Tageldin indicated no relevant financial relationships.
Asra Batool indicated no relevant financial relationships.
Ahmad Abulawi, MBBS1, Hala Abdelwahab, MD2, Omar Tageldin, MD2, Asra Batool, MD2. P1836 - Primary Small Cell Carcinoma of the Liver. Yes It Exists!, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.