Hemorrhagic metastasis to brain from a primary mucoepidermoid carcinoma of esophagus

Yatish Agarwal; Sagar Tomar; Amit Kumar; Avneet S Chawla; Vinod Gupta; Poonam Khambra

doi:10.4103/astrocyte.astrocyte_3_17

CASE IN POINT - CLINICS IN GASTROINTESTINAL ONCO-RADIOLOGY

Year : 2016 | Volume : 3 | Issue : 3 | Page : 165-167

Hemorrhagic metastasis to brain from a primary mucoepidermoid carcinoma of esophagus

Yatish Agarwal¹, Sagar Tomar¹, Amit Kumar¹, Avneet S Chawla², Vinod Gupta³, Poonam Khambra⁴
¹ Department of Radiodiagnosis, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
² Department of Surgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
³ Gastroenterology Unit, Department of Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
⁴ Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India

Date of Web Publication

27-Feb-2017

Correspondence Address:
Dr. Yatish Agarwal
Department of Radiodiagnosis, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/astrocyte.astrocyte_3_17

Abstract

Primary esophageal cancers rarely metastasize to the brain. On the rare occasion that such an event occurs, the histological cell type is mostly a squamous cell carcinoma. These brain metastases are picked up on contrast computed tomography typically as multiple ring enhancing hypodense lesions. In the histological spectrum of esophageal cancers, a mucoepidermoid carcinoma is rather rare. The possibility of it metastasizing to the brain is rarer still. That such a metastatic lesion be hemorrhagic is truly atypical, yet this case in point uncovers such an event, where an upper esophageal mucoepidermoid carcinoma in a 55-year-old male led to a hemorrhagic metastasis in the vermis of the cerebellum.

Keywords: Hemorrhagic brain metastasis, metastasis to cerebellar vermis, mucoepidermoid esophageal carcinoma, unsteady gait

How to cite this article:
Agarwal Y, Tomar S, Kumar A, Chawla AS, Gupta V, Khambra P. Hemorrhagic metastasis to brain from a primary mucoepidermoid carcinoma of esophagus. Astrocyte 2016;3:165-7

How to cite this URL:
Agarwal Y, Tomar S, Kumar A, Chawla AS, Gupta V, Khambra P. Hemorrhagic metastasis to brain from a primary mucoepidermoid carcinoma of esophagus. Astrocyte [serial online] 2016 [cited 2023 May 28];3:165-7. Available from: http://www.astrocyte.in/text.asp?2016/3/3/165/200997

Introduction

Metastatic brain tumors are the most common type of brain tumors found in adults. Such brain metastases (BMs) occur in approximately 25–35% of malignancies.^[1] The most common primaries are in the lung (48%) and breast (15%).^[2] BM in esophageal cancers is rare, and the incidence is no more than 0.5–4.8%.^[3],[4] Little data exists relating to the incidence of BM in different histological types of esophageal cancers. However, because squamous cell carcinoma is the predominant type, it is found to be the primary tumor histology in more than 95% of esophageal carcinoma patients with BM.

Primary mucoepidermoid carcinomas (MEC) of the esophagus are rather rare. They account for only 0.05–2% of all primary esophageal carcinomas.^[5] A thorough search of English literature revealed no instance where a primary MEC had led to a hemorrhagic BM.

Case Report

A 55-year-old male presented with progressive dysphagia both for liquids and solids, significant weight loss, and hoarseness of voice 10 months ago. The upper gastrointestinal (UGI) endoscopy conducted at the time revealed a 5-cm long endoluminal growth, 22 cm from the upper central incisor, causing a significant luminal narrowing. The endoluminal growth was biopsied. On histopathology, a pleomorphic tumor of epithelial origin with acinar formation and mucin production and hemorrhagic cells was demonstrated with a band of intermediate cells. Some squamoid cells, eosinophils, and variable-sized nuclei were also found in the background [Figure 1]. The histological findings paved the diagnosis for MEC, albeit a rare cell type among esophageal cancers.

Figure 1: H and E stain of biopsy specimen from the esophageal lesion. Pleomorphic tumor of epithelial origin with acinar formation and mucin production and hemorrhagic cells was demonstrated with a band of intermediate cells. Some squamoid cells, eosinophils, and variable-sized nuclei were also found in the background.

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Contrast-enhanced computed tomography (CECT) of the thorax conducted at the time demonstrated a heterogeneously enhancing mass measuring 10 cm × 5 cm × 3.2 cm (craniocaudal × anteroposterior × transverse diameter) in the upper and mid-thoracic esophagus extending from the upper border of first dorsal (D1) vertebra to the lower border of the fifth dorsal (D5) vertebra. The lesion had significantly narrowed the esophageal lumen, infiltrated into the posterior surface of trachea, and was found abutting the posterior surface of the right thyroid lobe with a loss of fat planes. Staging work-up revealed metastatic mediastinal lymphadenopathy (>7 nodes with short axis diameter >10 mm), indicating a stage T4b N3 M0 esophageal cancer [Figure 2]a,[Figure 2]b,[Figure 2]c.

Figure 2: (a) Contrast-enhanced axial image of the thorax (mediastinal window). Irregular circumferential thickening of the esophageal wall with endoluminal projection of the growth. Note the loss of fat planes between the tumor and the posterior wall of trachea. (b) Contrast-enhanced coronal reformatted image of the thorax (mediastinal window). Heterogeneously enhancing soft tissue density mass in the lumen of mid esophagus with severe luminal narrowing. (c) Contrast-enhanced mid-sagittal reformatted image of the thorax (mediastinal window). Large esophageal mass extending from D1–D5 vertebra with loss of fat planes with the the posterior aspect of trachea. The cerebellum demonstrates multiple hyperdense lesions portraying the hemorrhagic metastasis.

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Because the growth was unresectable, the patient was started on radiotherapy. The patient, however, despite receiving radiotherapy, developed newer symptoms of unsteady gait and poor balance producing difficulty in walking and standing 10 months later. A CECT brain demonstrated well-defined discrete hyperdense lesions in the vermis of the cerebellum with mild surrounding edema, diagnostic of hemorrhagic metastasis [Figure 3]a,[Figure 3]b,[Figure 3]c,[Figure 3]d. A simultaneous CECT thoracic study found no change in the size and extent of the primary esophageal growth or the mediastinal lymph nodes in comparison to the older CT performed 10 months ago, thus implicating the radioresistant nature of the tumor.

Figure 3: (a, b) Noncontrast CT axial sections of the posterior fossa of the brain. Ill-defined hyperdense lesions (hemorrhagic metastasis) with a minimal perilesional edema in the vermis producing a minimal compression of the 4th ventricle. (c, d) Contrast-enhanced CT axial sections of the posterior fossa of the brain. Ill-defined hyperdense lesion (hemorragic metastasis) with minimal perilesional edema involving the superior and inferior vermis without a significant contrast enhancement.

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Discussion

Esophageal cancer is the fifth most common cause of cancer-related deaths in men and the eighth leading cause of cancer mortality in women worldwide.^[6] Because of its extremely aggressive nature, it carries a poor prognosis and has a fatal outcome in the great majority of cases.^[1],[2] Histologically, it typically occurs in one of the two forms, i.e., squamous cell carcinomas (SCCs) arising from the stratified squamous epithelial lining of the organ and adenocarcinomas affecting columnar glandular cells that replace the squamous epithelium.^[7] Sarcomas and small cell carcinomas generally represent less than 1–2% of all esophageal cancers.^[8],[9] On rare occasions, other carcinomas, melanomas, leiomyosarcomas, carcinoids, and lymphomas may develop in the esophagus.^[10]

Distant metastases have been reported at initial presentation in 20–30% of patients with esophageal cancer, mostly in the liver (35%), followed by the lungs (20%), bones (9%), adrenal glands (5%), and, rarely, peritoneum and brain.^[11],[12] However, metastasis to the brain is very rare with a reported incidence of between 0.5% and 4.8%,^[3] with ring enhancing metastatic lesions mostly described.

More common in the salivary and lacrimal glands, primary MECs of the esophagus are rather rare, and account for 0.05–2% of all the cases of primary esophageal carcinoma.^[5] Of obscure origin,^[13] the hypothesis that these tumors arise from the esophageal glandular or ductal epithelium ^[14] is supported by their submucosal location, microscopic findings of normal stratified squamous epithelium overlying the MEC,^[15] and the common embryological derivation of esophageal and salivary glands.^[16] Histologically, they are a diffuse mixture of mucus-secreting glandular, intermediate, and epidermoid cells. They strike males in the 6^th decade of life and typically affect the middle and lower third segments of the esophagus.^[16],[17] Not recognizable clinically, they are diagnosed on the basis of the histopathologic findings, and it is not uncommon for them to be labeled as SCCs or adenocarcinomas.^[16] Traditionally, MEC has been divided into low, intermediate, and high grade types. Distant metastasis and lymphadenopathy is more commonly associated with high-grade MEC.^[18] Metastases from such tumors most frequently bring out the squamous component of the carcinoma.^[19]

Lymph nodal metastasis and surgery are independent prognostic factors; however, the prognosis remains dismal. Characterized by higher rate of recurrence and metastasis, surgery remains the only viable treatment for early esophageal MEC. Surgical treatment provides better palliation and a reasonable survival time and is preferred for patients with resectable disease who are physiologically fit enough to undergo surgery.^[15] These tumors show a poor response to radiotherapy and chemotherapy, with a median survival rate of 1–2 years.^[20]

Conclusion

The case in point is a combination of unique rarities. While MEC in itself is a rare tumor of the esophagus, the occurrence of an incident BM is still rarer. However, rather than the metastasis manifesting in the classic manner of multiple ring-enhancing hypodense lesions in the brain, it was peculiar that it was hemorrhagic in nature. A morphologic connotation of an advanced disease, it may well construe a terminal event in the temporal progression of the disease due to the radioresistant and chemoresistant nature of the tumor.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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