Esophageal adenocarcinoma with metastatic skin nodules and Budd–Chiari syndromeS Singh, P Bansal, A Arora, A Goel
Department of Medicine, Lady Hardinge Medical College and Dr. Ram Manohar Lohia Hospital, New Delhi, India
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/jpgm.JPGM_1319_20
Source of Support: None, Conflict of Interest: None
A 28-year-old female with no known comorbidities presented to the medicine outpatient department with gradually increasing abdominal distension for 2 months, vomiting and dysphagia to solids for the past 1 month, anorexia and weight loss. She also complained of skin lesions on the abdomen of recent onset. Her physical examination revealed pallor, multiple lymph nodes in the left supraclavicular fossa which were hard in consistency. Her abdominal examination revealed distended abdomen, with multiple dilated veins over the upper part of abdomen [Figure 1]a. She had multiple erythematous, firm, and tender nodules over the lower abdomen [Figure 1]b. On palpation of the abdomen, there was massive splenomegaly and fluid thrill.
Laboratory studies revealed mild anemia, normal liver and kidney function tests. Diagnostic paracentesis was done which revealed 500–600 cells/mm3 with 60% polymorphs, and a serum ascitic-albumin gradient of <1.1. Abdominal ultrasound revealed mild hepatomegaly, gross ascites with splenomegaly, and prominent portal vein with mesenteric and infra-umbilical collaterals, with no color flow in right hepatic vein suggestive of Budd–Chiari syndrome (BCS). The patient was worked up for possible etiologies of BCS including hypercoaguable states which were not detected. The patient underwent biopsy of the skin nodules and supraclavicular lymph nodes. The histopathological features of both the biopsies were consistent with metastatic adenocarcinoma with immunohistochemistry markers positive for cytokeratin (CK) 7 and CK 19 suggestive of a possible primary in upper digestive or pancreato-biliary tract.
The patient underwent contrast-enhanced computed tomography (CT) of the chest and abdomen which showed normal liver, multiple peripherally enhancing necrotic lymph nodes in pretracheal, paratracheal, preaortic region, paraesophageal and bilateral hilar region, thickening of mid and lower esophagus, portal hypertension with splenomegaly, and collateral formation with attenuation in hepatic veins confirming BCS, and gross ascites. The patient was referred to gastrosurgical oncology department with a provisional diagnosis of esophageal adenocarcinoma, with evidence of BCS on sonography and CT, for further management. The diagnosis was confirmed following an esophagogastroduodenoscopy and biopsy.
Metastases to the skin from internal tumors are uncommon. They indicate an advanced stage of a neoplasia, or its recurrence following treatment, or may be the first manifestation of a malignancy. The incidence of cutaneous metastases varies from 0.7% to 9% of all cases of malignant disease. They are the result of direct invasion or local or distant spread of a primary malignancy.
Cutaneous metastases in women are seen with breast cancer, and malignancy of the intestines and lung. In men, the most common primary is bronchogenic carcinoma, followed by malignancy of the stomach and larynx. Single or multiple nodular lesions are the most common presentations of cutaneous metastasis. The morphological features of the primary tumor are often reflected in the cutaneous deposits and thus, skin biopsy provides an inexpensive method to the clinician for confirming the possible primary site of the malignancy. Adenocarcinomas have been identified as the most common tumor type on histopathologic examination of metastatic skin lesions.
BCS is a rare disease characterized by obstruction of hepatic venous outflow. The obstruction may be thrombotic or nonthrombotic. The syndrome is considered primary if the obstruction is due to endoluminal venous lesion-like thrombosis. In secondary BCS, the cause originates from neighboring structures such as extrinsic compression or tumor invasion. Malignant neoplasms account for 10% of the cases. Patients present with ascites and other symptoms related to long-standing portal hypertension. Hepatic veins without flow signal, and collateral hepatic venous circulation on Doppler ultrasonography of the abdomen, are indicative of BCS, as was seen in our patient.
This case is worth highlighting as a young lady with no prior diagnosis of malignancy presented with cutaneous nodules together with BCS, and was detected to have metastatic adenocarcinoma of the esophagus. This is a rare first presentation of a malignancy.
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