Woman, in 50's, developed ongoing and worsening ear and same sided facial pain. The diagnosis of trigeminal neuralgia was made. Was treated conservatively, i.e., medications, injections and ultimately, microvascular decompression, without evidence of arterial compression of the nerve. With ongoing symptoms, particularly ear pain, patient underwent a second decompression, and a third procedure, a nerve resection, along with radiofrequency ablation. These procedures, along with ongoing conservative treatments, such as Botox, also failed to improve the patient's symptoms. The diagnosis of trigeminal neuralgia and the aforementioned treatments occurred over a four year period. During this period, the patient did have multiple, non-contrasted MRI and CT studies. The radiology reports do not describe a mass. Of note, there are references of hypervascular changes within the ear.
The patient was at an unrelated dermatology appointment when the dermatologist noted an abnormality within the ear canal. A biopsy was taken and pathology reported as basil cell carcinoma. (the pathology is being reviewed separately) At that point, the patient was referred to a different ENT for surgery. However, this ENT encountered a more significant and suspicious mass. The procedure was terminated, and initial pathology review was concerning for ceruminous carcinoma, microcystic adnexal carcinoma and syringoid eccrine carcinoma with positive margins and perineural invasion. At that point, the patient was referred to a nearby academic based ENT practice. Repeat imaging revealed a likely mass, and a second review of the pathology revealed ACC with mixed tubular and cribform findings.
The patient has subsequently undergone multiple neck and facial surgeries, including reconstructive procedures in an attempt to reduce the resulting and significant head, neck and facial deformities. The initial staging was ACC, pT4NO. While metastasis was initially negative, the patient has since developed lung metastasis.
I am looking for an ENT with a subspecialty in surgical oncology to evaluate the potential delay in diagnosing ACC. This includes issues such as the timing of obtaining repeat imaging studies, obtaining contrast v. non-contrast studies, and whether the overall clinical picture and consideration of tumor as the etiology of trigeminal neuralgia was adequately explored and/or ruled out.
Thank you for your consideration.
Files:
Q: What was the time frame between the imaging studies and evaluation with the different ENTs?
A: Initial Brain MRI in October of 2014; 2nd Brain MRI in January of 2015; Sinus CT in January of 2016; and Temporal Bone CT in September of 2017. Patient underwent surgery for the "presumed BCC" in July of 2018 leading to ACC dx.
Do you believe there might have been medical error?
Difficult to say if there was a medical error based on the information given. How was the diagnosis of trigeminal neuralgia arrived at? Was there imaging done prior to arriving at that diagnosis? What was that imaging? Contrasted imaging is usually preferred over non contrasted imaging unless there was some contraindication to using contrast such as renal insufficiency. If imaging was done and did not show any mass, then there was likely no error. If imaging shows a mass or no imaging was done before arriving at the diagnosis of trigeminal neuralgia, then there was an error.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
If an error was made as outlined above, and diagnosis was delayed for four years, then there was injury as likely the surgery and treatment would have been less invasive and possibly the oncologic outcome could have been better.
What makes you a good expert for this case?
I am a head and neck surgical oncologist at a major academic center with 12 years of experience after completion of training.
How often do you encounter cases similar to this one in your practice?
I deal with salivary gland tumors like this case several times a month.
Do you believe there might have been medical error?
The finding of unilateral facial pain is a tumor until proven otherwise. I am unsure as to the specialties of the initial evaluating providers - likely neurology, but in the face of many multiple types of procedures over 4 years, and evaluation for TMJ, and an exam of the ear canal would be indicated. Ear Canal cancer is extremely rare, but any type of persistent bleeding or growth in the canal would require a biopsy and any pathology would warrant a contrast film to evaluate. The issue one must weigh is that this is an extraordinarily rare cancer, and the retrospectoscope makes things look more obvious than they may appear on first glance.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
There was ultimately a delay in diagnosis, but given the type of lesion, its location, and no findings as to its pathologic grade, one cannot assess whether this would have been a likely outcome if it was resected previously. I believe trigeminal neuralgia is more of a diagnosis of exclusion, and frequently lumped in with other findings like migraine.
What makes you a good expert for this case?
You need two different types to be most effective, one who is a more general ENT or neurology that can talk about the differential of her facial pain and an oncologist or ENT Head and Neck surgeon who can talk about his/her experience with these tumors and prognosis if caught early vs stage she was found in. Again most of these, especially with perineural spread tend to recur and disfigure. I can speak on the more general ENT part and facial pain
How often do you encounter cases similar to this one in your practice?
These are extremely rare and most offices a once in a career type of case.
Do you believe there might have been medical error?
The central questions are: Who, meaning what type of Physician or Provider, was directing the evaluation to this woman's "ear and facial pain.? Was the ear examined by an ENT Surgeon and/or an Otologist? When, how often? Was TMJ Dysfunction also diagnosed, thus confounding the evaluation? Why was imaging ordered? The Radiologist Consultant can make a determination of whether or not to use contrast for the diagnoses being considered. Did the patient have iodine allergy? How were "hypervascular changes noted if no contrast? If these were not the case, then perhaps the diagnosis was delayed.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
If the final definitive diagnosis is as listed, Adenoid Cystic Carcinoma, presumably of a Ceruminous Gland of the External Ear Canal (and not Parotid Gland?) that presented with ear and facial pain, then the process already had perineural invasion at time of presentation, thus was locally spread, if not distantly spread given the vascularity of the head and neck region.
What makes you a good expert for this case?
38 years of both Academic and Clinical Otolaryngology and now Attending Surgeon in Otology.
How often do you encounter cases similar to this one in your practice?
As described, this is a rare case, a very rare case indeed.
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