Ear infection is often ignored .Chronic ear disease happens because of refusal to treat acute or subacute ear infection on time.The worse part is surgery when mentioned to the patient,is the fastest way for poor patient follow up.Unfortunately surgery is often the mainstay of chronic ear disease

 The Story

A 52-year-old woman had intermittent ear discharge since childhood and gradually worsening hearing.She was advised surgery in 2020 which she finally agreed to get done in 2025.

Routine ear surgery was planned to repair the eardrum. After surgery, her hearing improved — but her biopsy report  revealed something unexpected: a rare tumor called Biphenotypic Sinonasal Sarcoma (BSNS), found in her middle ear.

 What Is Biphenotypic Sinonasal Sarcoma?

It’s a rare, slow-growing cancer that usually starts in the sinuses or nasal passages. It shows two different cell types under the microscope — one resembling nerve cells and another resembling muscle cells.

The tumor’s cause is a genetic change (PAX3 gene fusion) that makes cells grow abnormally but slowly.

Why It’s So Rare

Most BSNS tumors are found in the nose or sinuses.

Finding one in the middle ear is almost unheard of.

Symptoms like ear discharge and hearing loss mimic chronic infections, making diagnosis tricky.

 How We Found It

During surgery, a small tissue sample (biopsy) was sent for testing. Under the microscope, pathologists found spindle-shaped cells — and special tests called immunohistochemistry (IHC) showed specific markers (S100 and SMA).

This confirmed it was a biphenotypic sinonasal sarcoma — a low-grade tumor that rarely spreads but can come back locally.

 Treatment & Outlook

Surgery is the main treatment.Patient was referred to a specialized higher centre both Government and Private for further opinion regarding best course of management.

Radiotherapy may be used if the tumor can’t be fully removed.

Regular follow-up is important to monitor for recurrence.

The good news: it’s low-grade, meaning it grows slowly and is often manageable if detected early.

🌿 Takeaway for Readers

 1. Chronic ear infections need evaluation — sometimes they hide more complex issues.

2. A biopsy during surgery can uncover rare diseases early.

3. Early diagnosis and expert pathology make all the difference.

I first wrote about this case report here.

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