Merkel Cell Carcinoma

Left: Merkel cell carcinoma recurrence a few months after excision. Right: Progressive increase in size of recurrence over a few months. (Dr. Noorali Bharwani)

82 year-old male nursing home resident presented with a subcutaneous lump on the left knee for three to six months. Medical history includes head injury with intra-cranial bleed due to a fall, resulting in permanent physical disability and cognitive deficit.

The lump was about four cm in diameter. The patient and family requested excision biopsy as the patient kept drawing their attention to the lump that progressively turned red, as if it was getting inflamed. My first impression was that this was not a lipoma but probably an inflamed sebaceous cyst. It was excised under local anesthetic without any complications.

Pathology:

Merkel cell carcinoma of the skin, 4 cm in size. Peripheral and deep margins were extensively involved with lymphovascular invasion.


Merkel Cell Carcinoma (MCC)

Merkel cell carcinoma, a rare type of skin cancer, usually appears as a flesh to bluish-red colored nodule on sun-exposed areas, like the face, head and neck.

Merkel cell carcinoma is usually found in older people. Around 80% are caused by Merkel cell polyomavirus. Exposure to sunlight and a weak immune system increase the chance of developing MCC.

The tumor is locally invasive and also spreads rapidly through the body. Early diagnosis and treatment lowers the chance of metastasis.

Since the lesion has no distinguishable features from other skin cancers, the first treatment is surgical excision. Once the pathology report comes back the lesion can be identified as Merkel cell carcinoma. Further therapies like lymph node biopsy, radiation or chemotherapy can be considered if needed, to prevent metastasis and recurrence.


In this case, no further investigation or treatment were planned, as the patient’s quality of life would be extremely poor should he survive recurrence. The family decided it was time to let nature take its course.

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Parotid Gland Abscess

Abscess over the left parotid gland area. (Dr. Noorali Bharwani)

46 year-old female presented with a two-week history of an abscess over the left parotid area. The patient reports an abscess in the same location 18 months prior which she had aspirated. She has a medical history of Sjögren’s syndrome.

The patient had finished a course of amoxicillin-clavulanic acid and then was started on a course of a cephalosporin. Incision and drainage was done under local anesthetic and a large amount of pus was drained. The patient healed well.

Culture: Streptococcus anginosus

Streptococcus anginosus is part of the human bacteria flora, but can cause diseases including brain and liver abscesses under certain circumstances.” (Wikipedia)

Pathology: Showed no parotid tissue. There was acute-on-chronic inflammation of subcutaneous tissues.


Salivary Gland

Infection: parotitis, sialandenitis

Symptoms – One-sided salivary gland swelling. Fever and pain will accompany the swelling.

Pathogens – Typically normal bacteria found in the mouth. Viral infections such as mumps often affect the salivary glands. Mumps most often involves parotid salivary gland. Mumps is a rare problem today because of the MMR vaccine.

Dehydration and malnutrition raise the risk of getting a bacterial infection.

Sjögren’s syndrome

This is a chronic autoimmune disease in which cells of a person’s immune system attack the salivary and other moisture-producing glands, leading to dry mouth and eyes. About half of people with Sjögren’s syndrome also have enlargement of the salivary glands on both sides of the mouth, which is usually painless.

Treatment

In some cases, no treatment is needed.

Antibiotics for fever or pus drainage, or if the infection is caused by bacteria. Antibiotics are not useful against viral infections.

Surgery or aspiration to drain abscess.

Self-care steps that can be done at home to help with recovery:

  • Practice good oral hygiene. Brush teeth and floss well at least twice a day. This may help with healing and prevent infection from spreading.
  • Rinse mouth with warm salt-water rinses (1/2 teaspoon of salt in 1 cup of water) to ease pain keep the mouth moist.
  • Stop smoking.
  • Drink lots of water and use sugar-free lemon drops to increase the flow of saliva and reduce swelling.

Prognosis

Most salivary gland infections go away on or are cured with treatment. Some infections will return. Complications are uncommon.

Complications

  • Abscess of salivary gland
  • Infection returns
  • Spread of infection (cellulitis, Ludwig’s angina)

Prevention

In many cases, salivary gland infections cannot be prevented. Good oral hygiene may prevent some cases of bacterial infection.

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Pilomatricoma

Pilomatricoma before and after excision. (Dr. Noorali Bharwani)

A 47 year-old male presented with a three-month history of a lump below the right eyelid. It was slowly getting bigger. The lump was excised under local anaesthetic.

Pathology report

Specimen

Cyst right lower eyelid.
The specimen consists of a tan and brown irregularly shaped piece of skin measuring 1.3 x 0.5 x 0.2 cm. Attached is an underlying cyst-like structure measuring 1.0 x 0.5 x 0.5 cm. The cyst-like structure is extremely friable. The margins are marked with blue ink.

Diagnosis

Consistent with proliferating pilomatricoma


Pilomatricoma

Pilomatricoma is a benign cystic new growth.

Clinically, pilomatricoma usually presents in young individuals as a solitary cutaneous nodule with an average size of one cm and rarely exceeds 2 cm in diameter.

Proliferating pilomatricoma was first described in 1997.

The lesions are usually situated mostly on the head and neck.

The differential diagnosis includes classical pilomatricoma, pilomatrical carcinoma, and basal cell carcinoma.

These neoplasms should be excised with adequate surgical margins, and careful follow-up examinations are strongly recommended.

Proliferating pilomatricoma can occur at younger ages and should be considered in the differential diagnosis of solitary lesions in adults and children, even if the lesion is rapidly growing.

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Shingles Vaccine may be Viewed as a Quality of Life Vaccine

An example of shingles. (iStockphoto/Thinkstock)


A man with shingles of the face.

Shingles is caused by chickenpox virus called varicella zoster virus. The first indications that chickenpox and shingles were caused by the same virus were noticed at the beginning of the 20th century.

The incidence of shingles is mainly in adults. There are approximately four cases per 1000 population per year and a lifetime risk of 20 to 30 per cent.

Chickenpox generally occurs in children. Once the child gets over the illness the virus does not disappear from the body. Virus can settle down in one of the nerve cell bodies and lay dormant for many years.

When your resistance is low and this can be due to any reason, the virus may break out of the nerve cell and travel down the nerve causing viral infection of the skin in the area supplied by that nerve. This can happen decades after the chickenpox infection. Exactly how the virus remains latent in the body, and subsequently re-activates is not understood.

Shingles starts with burning pain, itching and tingling followed by painful rash and blisters in the area supplied by the affected nerve. The pain and rash most commonly occurs on the torso, but can appear on the face, eyes or other parts of the body. If the nerve to the eye is involved then a person may suffer loss of vision. It usually affects one nerve on one side of the body.

The rash and blisters heal within two to four weeks but some sufferers experience residual nerve pain for months or years. This condition is known as postherpetic neuralgia. About 20 per cent of patients with shingles suffer from this.

If the diagnosis of shingles is made early then it helps to start antiviral medications within 72 hours of the appearance of the rash. This reduces the severity and duration of the illness. The antiviral medications should be used for seven to ten days. The blisters crust over within seven to ten days, and usually the crusts fall off and the skin heals. But sometimes after severe blistering, scarring and discolored skin remains.

Until the rash has developed crusts, a person is extremely contagious. During the blister phase, direct contact with the rash can spread the virus to a person who has no immunity to the virus. This newly infected individual may then develop chickenpox, but will not immediately develop shingles.

Since 2008-2009, a vaccine for shingles is available for adults age 60 and over. The vaccine is used to boost the waning immunity to the virus that occurs with aging. The effectiveness of the vaccine is about 60 per cent. It is kind of a “quality of life” vaccine. It does not prevent death from shingles (an extremely rare event) but does help with postherpetic neuralgia (pain).

Booster doses of the vaccine are not recommended for healthy individuals. The efficacy of protection has not been assessed beyond four years and it is not known whether booster doses of vaccine are beneficial. This recommendation may need to be revisited as further information becomes available.

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Use of MRI in Evaluating Breast Lumps

A woman receiving a scan. (iStockphoto/Thinkstock)


A woman with locally advanced breast cancer.

The traditional way to assess a breast lump is to take a history, do a physical examination, do a fine needle aspiration cytology (examination of a breast lump aspirate under a microscope), mammogram and/or ultrasound, core biopsy under ultrasound control and finally, if there is no satisfactory answer then do a surgical biopsy.

A surgical biopsy gives us a definitive answer. But there are drawbacks to sending every patient with a breast lump for surgery. To start with it causes severe anxiety. You have to take a day off work. It requires local or general anaesthetic. There may or may not be postoperative complications like bleeding, bruising, discomfort, infection and pain.

On a long term basis, surgical biopsy will leave you with a scar and may be another lump which may be just a scar tissue but could be suspicious for cancer. Then you have to go through the whole process all over again.

Is there anything else we can do before going for surgery to make sure that there is no cancer in the breast?

You can ask for a second opinion. If all investigations are negative then there is a less than five per cent chance that cancer has been missed. In that case, we can leave the lump alone and provide follow up care with clinical examination and mammography or ultrasound, on a case by case basis. Sometimes a patient will ask for MRI.

MRI (magnetic resonance imaging) is a procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. MRI does not use any x-rays.
MRI is not available for routine screening. It is expensive and requires specialized equipment and personnel with good solid training to read the images. Hence, it is available in bigger cities only and is not covered by government insurance plans. MRI is more often used for breast imaging in the US than Canada because of the prevalence of private health care.

MRI is sensitive to small abnormalities in breast tissue. MRI also has limitations. For example, MRI cannot detect the presence of calcium deposits, which can be identified by mammography and may be a sign of cancer.

The value of breast MRI for breast cancer detection remains uncertain. And even at its best, MRI produces many uncertain findings. Some radiologists call these “unidentified bright objects,” or UBOs.

In women with a high inherited risk of breast cancer, screening trials of MRI breast scans have shown that MRI is more sensitive than mammography for finding breast tumors. Screening studies are ongoing.

Breast MRI is not recommended as a routine screening tool for breast cancer. However, for women at high risk, women with previous breast cancer, MRI can be useful in certain circumstances.

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Meckel’s Diverticulitis can Mimic Acute Appendicitis

A girl with lower abdominal pain. (iStockphoto/Thinkstock)


A specimen of acutely inflamed Meckel’s diverticulum.

It was nine o’clock in the evening. I was on-call for the general surgery group. After a busy day at the office and the hospital, and after a late supper, I had just sat down to watch some news on TV. The phone rings. My wife answers. She says to me, “It’s for you, honey. It’s the hospital emergency.”

The ER physician had just examined an ill looking seven-year old boy with right sided abdominal pain, nausea, vomiting and fever. The ER doctor wanted me to come and give a surgical opinion. The question I will be asked in ER is, “Does this boy have an acute appendicitis and does he need to go to OR for surgery this evening?”

After going through the boys history and physical examination, I came to the conclusion that the kid was quite sick with abdominal signs of acute appendicitis. Possibly perforated appendicitis and peritonitis. He was dehydrated. Intravenous fluids were given, preoperative antibiotics were given and he was taken to OR.

In the OR, as soon as the kid’s belly was opened, a large amount of purulent fluid poured out. The appendix looked normal. There was a hole in the small bowel where it meets the cecum (beginning of colon) where the appendix is located. The appendix, the terminal part of the small bowel and cecum were all stuck together due to the acute inflammation. To stop the leak from the small bowel, there was no choice but remove the terminal part of the small bowel, appendix and the cecum (called right hemicolectomy).

Postoperatively the child did very well. He went home nine days after surgery. Pathology of the specimen showed normal appendix, Meckel’s diverticulum with gastric mucosa with ulceration and perforation in the adjacent small bowel and peritonitis. Acid secretion from the gastric mucosa in the diverticulum had caused the ulceration and perforation.

A Meckel’s diverticulum is a true congenital diverticulum (bulge) in the small intestine present at birth. It is a vestigial remnant of the omphalomesenteric duct (also called the vitelline duct or yolk stalk), and is the most frequent malformation of the gastrointestinal tract.

It was first described by Fabricius Hildanus in the sixteenth century and later named after Johann Friedrich Meckel, who described the embryological origin of this type of diverticulum in 1809.

It is not that common. It is anti-mesenteric (on the free margin of the small bowel). For a medical student, the best memory aid is the rule of 2s: two per cent of the population, two feet from the ileocecal valve, two inches in length, two per cent are symptomatic, two types of common ectopic tissue (gastric and pancreatic), two years is the most common age at clinical presentation and two times more boys are affected.

Most people who are born with this have no symptoms. The most common presenting symptom is painless rectal bleeding such as black offensive stools, followed by intestinal obstruction, volvulus (torsion) and intussusception where a part of the intestine has invaginated into another section of intestine, similar to the way in which the parts of a collapsible telescope slide into one another. Over the years, I have seen examples of each one of the complication.

If a patient has symptoms and clinical diagnosis is not clear then it is worth doing a Meckel’s scan using technetium-99m (99mTc). This scan detects gastric mucosa; since approximately 50 per cent of symptomatic Meckel’s diverticula have ectopic gastric or pancreatic cells contained within them. Treatment is surgery.

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Gallstones can be a Source of Pain and Misery

Gallstones (iStockphoto/Thinkstock)

Clinical - Gall bladder and Gallstones
Left – gallbladder with stones. Right – 952 gallstones from my 35-year-old male patient who presented with chelecystitis, pancreatitis and common bile duct stones.

How many gallstones does it take to get symptoms?

Just one. The size and shape of the gallstone does not matter. The more stones you have the more risk of getting symptoms. There are many patients who have gallstones and have no symptoms. These patients do not require surgery until they are symptomatic or they are moving to a country where the health care system is not very reliable.

What is the world record for the number of gallstones removed from a single gallbladder?

According to a Medical Post (October 6, 1987) clipping I have on file, two British surgeons claimed a world record for the the number of gallstones removed from a single gallbladder – 23,530. This was in an 85-year-old woman who presented with severe colicky central abdominal pain. The gallstones are now in the pathology museum at St. Thomas Hospital medical school in London, England. I wonder who counted them all.

What is my record?

On October 8, 1987 I removed a gallbladder from a 35-year-old Saskatchewan man which contained 952 gallstones (see picture). I counted these myself! The man presented with gallstone pancreatitis and jaundice. He had severe upper abdominal pain and vomitting. He also had stones in the common bile duct. This is the duct which transports bile from the gallbladder to the intestine to help us digest food.

Who is the youngest patient to have gallbladder removed for gallstones?

According to Guinness World Records, an American child, Danylle Otteni was five years and six months old at the time she had her gallbladder and gallstones removed in Philadelphia, Pennsylvania, USA, on 14 October 2007. If I remember correctly, my youngest patient was around 14 years old.

Gallstones are very common in Western countries. Probably due to our diet which is high in refined and processed food. There are other factors which can contribute to gallstone formation. But we do not know exactly why some people have gallstones. There is some chemical imbalance in the bile which precipitates cholesterol to form a nucleus for a stone. On the basis of their composition, gallstones can be divided into the following types: cholesterol stones, pigment stones and mixed stones.

The treatment for symptomatic gallstones is surgery. It is the commonest elective general surgical procedure we do.

Once, the surgical procedure of choice was open cholecystectomy. In fact, Carl Langenbuch carried out the first cholecystectomy in 1882. Patients who undergo open cholecystectomy have a long surgical incision in the abdominal wall, have to stay in the hospital three to five days, consume fair amount of pain killers for post-operative pain and the recovery time at home is three to six weeks.

Things have changed in the last 15 to 20 years. Now most patients undergo laparoscopic cholecystectomy for symptomatic gallstones. Laparoscopic cholecystectomy was first performed in France in 1987. There are four tiny incisions in the abdominal wall, the hospital stay is usually overnight, the amount of pain killers required after surgery is minimal and the recovery time at home is usually less than one week. That is called progress.

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