Famous Patients, Famous Operations, 2004, Part 3 - The Case of the Orphan With a Rare Disease

Albert B. Lowenfels, MD

Disclosures

November 04, 2004

Introduction

The patient was a 51-year-old professional athlete with a chief complaint of hoarseness and headache.

Present Illness: The patient had been previously healthy until onset of present illness. Headache was localized to the left side; it seemed to be most severe in the retroorbital region. Initially, the pain was thought to be sinusitis, or perhaps an infected tooth, but the pain persisted even after extraction of several teeth. Pain persisted for a few months without any clear diagnosis. At the time of hospitalization, 3 months after onset of illness, the patient had persistent headache, hoarseness, and pain in the left shoulder. Physical examination at the time of initial hospitalization revealed a left Horner's syndrome and paralysis of the left vocal cord.

Past Medical History: The patient had been generally healthy. There was 1 hospitalization at age 30 for an "intestinal infection."

Physical Examination: The patient was a somewhat obese, muscular individual complaining of cough and headache. The main physical findings are described above.

Family History: The patient's mother and father died during middle age. The mother died of unknown causes, and the father died following a fight. The patient was 1 of 8 siblings; all but the patient and 1 sister died during childhood.

Social History: The patient was abandoned by his parents at age 7 and grew up in an orphanage. As an adult, the patient was a heavy drinker with frequent episodes of intoxication. He also smoked, used snuff, and had been overweight most of his life.

Hospital Course: Plain films of the skull taken soon after the initial hospitalization revealed a mass located at the base of the skull in the region of the foramen ovale and the jugular foramen. Despite the lack of a biopsy, the patient was treated with radiation therapy with slight improvement. However, several weeks after admission, the patient developed a mass in the neck that encased the carotid artery. He received additional radiation therapy along with hormonal therapy. During the 3-month hospitalization, the patient lost 80 lb.

Subsequent Course: The patient continued to have hoarseness and left-sided headaches. Dysphagia was noted. Several months after the onset of his initial symptoms, the patient received chemotherapy, with prompt but temporary improvement characterized by weight gain and diminished narcotic requirement. However, the pain and other symptoms gradually returned, requiring rehospitalization. The patient again received radiation therapy, including the implantation of radioactive gold seeds in the neck, but there was progressive spread of the disease leading to death at age 53, approximately 2 years after the initial onset of symptoms.

Who was our mystery patient?

  1. Jesse Owens

  2. Mickey Mantle

  3. Babe Ruth

  4. Joe Louis

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What is your diagnosis?

  1. Lung cancer

  2. Cancer of the larynx

  3. Nasopharyngeal cancer

  4. Tuberculosis of the throat

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What is Horner's syndrome?

  1. Constricted pupil, drooping eyelid, and facial dryness

  2. Contraction of the fingers from fibrous bands

  3. Bluish discoloration of the periumbilical skin

  4. Hyperirritability of the facial and other nerves

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What diseases can cause Horner's syndrome?

  1. Cancer of the lung

  2. Stroke

  3. Trauma to the neck

  4. Nasopharyngeal cancer

  5. All of the above

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What famous teammate developed another rare disease a few years before Ruth became ill?

  1. Lou Gehrig. At age 36, Lou Gehrig developed a rare neurologic disease -- amyotrophic lateral sclerosis, now often referred to as "Lou Gehrig's disease." The playing careers of both players overlapped, but Ruth's jealousy interfered with any friendship between these 2 famous Hall of Fame players. Ruth, who could be warm and generous, finally did pay tribute to the ailing Gehrig when poor health forced Gehrig to retire. The fact that 2 famous Yankee teammates developed rare diseases within a short time period is probably coincidental.

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What is the etiology of nasopharyngeal cancer?

  1. There are different types of nasopharyngeal cancer (NPC), but the most common form is undifferentiated NPC, which is associated with the Epstein-Barr virus.[2] Although rare in Europe and North America, it is endemic in southern China, Taiwan, the Philippines, and Vietnam. Babe Ruth never visited these endemic areas, although he did accompany the Yankees during a tour to Japan.When NPC occurs outside of endemic areas, it is usually squamous rather than undifferentiated and has been linked to both smoking and drinking. Ruth drank heavily, smoked, and used snuff. Both smoking and drinking have been linked to this tumor. Chow and coworkers[3] found that smoking 1 or more packs of cigarettes per day resulted in about a 5- or 6-fold increased risk of this tumor. Vaughan and coworkers[4] in a US study found that almost two thirds of squamous-type NPC tumors were attributable to the combination of smoking and drinking.

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How was Ruth treated?

  1. At first, because cancer was suspected even without a positive biopsy, Ruth received radiation therapy, with some symptomatic relief. The tumor eventually spread from the nasopharyngeal area to the neck, where a positive tissue biopsy was obtained. Attempted surgical removal proved impossible because the tumor involved the carotid artery.In 1947, because of persistent symptoms, Ruth agreed to an experimental treatment with pteroyl-triglutamic acid, known as "teropterin," a drug with antifolic acid properties. Within a few months, he improved markedly with shrinkage of the mass in the neck and improvement of voice and swallowing ability. But the remission was only temporary, and Ruth died in August 1948 from metastatic cancer originating in the nasopharynx.

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How would this tumor be treated today?

  1. The diagnosis of occult NPC cancers has become more accurate with newer diagnostic tools. A positron emission tomographic (PET) scan combined with a magnetic resonance imaging (MRI) or computed tomographic (CT) scan would surely have revealed the location of Ruth's cancer more accurately than a conventional x-ray of the skull. Because of its inaccessible location, radiation therapy and chemotherapy, rather than surgery, are still the most widely used therapeutic options. With the introduction of cisplatin, fluorouracil, vincristine, methotrexate, and other chemotherapeutic agents, 3-year overall survival rates have been reported to be around 87% for groups of patients treated with combined chemoradiation therapy.[5] But it seems unlikely that Ruth's tumor would have been cured, even with current therapy.

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