Jaw Tumor: Ameloblastoma

All “tumors” are not cancerous, but they can be severely disfiguring, obstructive and the need for a major surgical resection is necessary. Ameloblastoma is one of those tumors. Most of these tumors are totally benign, meaning the are not cancerous and the ability for them to metastasize to another part of the body is rare. A majority of these tumors originate in the jaw (mandible) and can grow very large and disfiguring, disrupting teeth and normal placement of the tongue in the mouth. Because these changes can severely effect the patients ability to eat and breathe, surgical resection is an absolute must.

These tumors originate from immature cells that are normally present in the jaw to produce tooth enamel. Although these tumors are benign, they can cause severe bone destruction.

Here are a few clinical photos of patients that have ameloblastoma. As you can see these tumors can really distort a patients face.

These tumors are the second most common tumor of the jaw (odontoma #1). They are slow growing and tend to be present in men and woman in between 30-50, although the can be present in children/ young adults. Ameloblastomas typically occur as hard painless lesions near the angle of the mandible in the region of the 3^rd molar tooth although they can occur anywhere along the alveolus of the mandible. Approximately 20 % of these tumors can occur in the maxilla (upper jaw).

Imaging

On imaging you can see how destructive a tumor like this is to the mandible. Some of these tumors have a multiloculated, cystic destruction of the bone.

Surgical resection of this tumor is the first step, but these tumors can have a high rate of recurrence. If possible, the surgeon will try to resect the entire tumor to minimize this. Below is a photo of total mandible resection for ameloblastoma surgical resection. The jaw is large and nodular distorting the teeth.

This is a close view of the surgical resection margin. You can clearly see where the tumor stops and the small (aprox 1cm) free margin of “normal” appearing mandibular bone. Because this tumor appears to have been completely excised, the rate of recurrence is less likely.

The most striking appearance of this tumor is on the cut sections. Here you can appreciate how desructive this tumor is to the normal solid bone of the mandible. It corresponds to the cystic destruction of the bone seen on imaging.

Reconstruction

Seeing such a gruesome surgical resection like this make you think the patient may be left severely deformed. Not true. The surgical reconstruction of these types of tumors is absolutely amazing! Often an “autograft” with a titanium plate is used which means it is the patients own bone. The bone is usually grafted from the patients own fibula (small lower leg bone) or rib.

This is an example of a patient who had an autograft of the rib, status post ameloblastomas resection.

Sexually Transmitted Disease You Don’t Want: Human Papillomavirus (HPV)

HPV is one of those STD’s you want to tell your kids about. There are multiple strains of this virus, each one causing nasty effects to the body, especially in girls. HPV stand for human papillomavirus and this virus can effect the skin and mucosa of the vulva, vagina, cervix, anus, scrotum, penis, lips, mouth and throat. There are many of strains of HPV and some have been identified that cause different effects on these areas of the body.

The curse of HPV: The “bad” strains of HPV (high risk) such as type 16 and 18 do not usually cause warts that a visible to the naked eye, however they can cause changes to the cells that can turn that tissue into cancer. The “good” strains of HPV (low risk) such as types 6 and 11 do not cause cancer, however they can cause nasty, disfiguring warts that range in size from the head of a pin to a cauliflower floret.

Most people infected with any strain of the HPV virus do not have visible warts- which makes this a dangerous STD. Obviously any person would stay away from a sexual partner with growths on their genitals. This virus can be spread with no visible warts.

What this looks like…

High risk HPV can cause nasty changes to cells microscopically. These changes greatly increase the risk of cancer. Pap smears are done at least annually on sexually active teens and adults to look for these abnormal changes caused by HPV 16 and 18. If atypical cells are seen the remainder of the tissue collected at the pap smear is sent for viral testing. The lab will alert the gynecologist if high risk HPV was detected in the patients specimen. Depending on the level of abnormality seen in the pap smear depends on the management of these patients. The patients can be under surveillance with repeat Pap smears or the gyno may decide to give the patient a biopsy to see exactly what is going on in the tissue of the cervix. If the patient has high risk HPV and moderate to severe atypical cells are seen on the biopsy, the patient will then be send for a cold knife cone biopsy or LEEP excision (Loop electrocautery excision procedure). These are pretty nasty surgical procedures in which they cut out a nice size chunk of the ecto and endocervical portions of the cervix.

A cone biopsy or LEEP is usually enough surgery to excise these lesions cause by high risk HPV. If atypical cells still persist or get more atypical then a hysterectomy will need to be performed. Back in the day, before clinicans and scientists were hip to HPV, cervical cancer was a leading cause of death in woman. Dr. Papanicolaou got hip to these changes in cervical cells and started performed to Pap smears to surveil woman. Since then, the incidence of cervical cancer has dropped significantly.

Low risk HPV on the other hand can form genital warts that can be seen on the genitals. The lesions can be large and disfiguring to the genitals. These lesions rarely lead to cancer.

Treatment for low risk HPV varies on extent of lesions and locations. Topical creams are sometimes used to boost the immune system into destroying HPV infected cells and killing off the warts. Other times cryosurgery (freezing) is used. Surgical excision of the warts is done, usually if warts are large and obstructive to the penis, vagina or anus.

This is what a surgical excision of genital warts can look like. A lesion like this is big to be on the genitals- about 4 cm or 1.5 inches. This is approximately the size of a strawberry.

Other areas of the body effected by the HPV virus include the anus, lips, mouth, tongue, throat and vocal cords. Wart like nodules can appear on these areas as well as precancerous changes to the cells of these areas. Remember oral and anal sex can still transmit these viruses!

Prevention of HPV

The HPV vaccine (Gardasil) is an awesome prevention of the HPV virus. Because there are so many strains of the HPV virus, they can not all be prevented with this vaccine. However, the most common strains of this virus are covered including high risk HPV (16 and 18) and low risk HPV (6 and 11).  This vaccine is only recommend at this time for girls under 26 years old, but it is being tested in boy/men since they can also be carriers of the virus and rarely can develop genital warts or genital cancer.

The most reliable prevention is safe sex or abstinence. Preach to your kids to use condoms!

Developmental Fetal Anomaly: Craniorachischisis Totalis

Neural tube defects are fairly uncommon, mild to severe congenital malformations resulting from complete or partial failure of the neural tube to close in the developing embryo. Neural tube defects include anencephaly and different degrees of spina bifida. Craniorachischisis totalis is the most extreme form of a neural tube defect with anencephaly and spina bifida. The fetus shows complete absence of the brain and calvaria and incomplete closure of the entire neural tube.

Approximately 4000 fetuses are affected yearly with a neural tube defect. 1/3 of them are lost during pregnancy because of spontaneous or elective abortion.  All infants with anencephaly are stillborn or die shortly after birth.

There is strong evidence that links these birth defects to a folate deficiency in the mother.  The incidence of anencephaly and spina bifida is usually higher in groups with lower socioeconomic status and at least half the cases of neural-tube defects can be prevented if women consumed sufficient amounts of the folic acid before conception and during early pregnancy. Few specific causes of neural tube defects have been recognized aside from folic acid deficiency, except for relatively rare sources of exposure such as maternal diabetes and maternal use of some antiepileptic drugs, such as valproic acid.

Though a fetus may have such a malformation that they’re unable to sustain life independently, the cells in other parts of the body do not know that and the rest of the fetus continues to grow normally. That is why such a catastrophic defect can be present in older fetuses and babies at term.

Ectopic Pregnancies…

A positive pregnancy test does not always ensure a normal intrauterine pregnancy. A pregnancy test bought at a drug store or one that is done at a physicians office using a patients urine is only testing for a hormone called hCG or human chorionic gonadotropin. This hormone being present in the urine is just telling the patient and/or the physician that the patient is pregnant. To confirm a pregnancy a quantitative hCG blood test is needed. This indicates 100% the presence of pregnancy hormone in the blood. This test also measures pregnancy hormone levels in the blood. In general the hCG levels will double every 72 hours. The level will reach its peak in the first 8 – 11 weeks of pregnancy and then will decline and level off for the remainder of the pregnancy. If the levels are tested and are too low or high, it can be indicative of a problem. Too low levels can mean there is an active miscarriage, blighted ovum (no embryo) or ectopic pregnancy. Too high levels can mean a molar pregnancy or multiple pregnancy.

Most pregnancies are intrauterine. Actually only 1% of all pregnancies are ectopic (outside the uterus).Ectopic pregnancy occurs when a fertilized egg attaches itself somewhere other than within the uterus. 98% of these ectopic pregnancies occur in the fallopian tube. The fallopian tubes are not designed to hold a growing embryo; the fertilized egg in a tubal pregnancy cannot develop normally and must be treated. 2% of ectopic pregnancies occur in the ovary, cervix, or are intraabdominal. The fetus is not typically viable but rare cases a baby has been delivered from an abdominal pregnancy.

Ectopic pregnancies are most common in woman age 35-44. It is more frequently found in woman who have some kind of irregularity of the fallopian tube. Fallopian tubes can be congenitally small. They can also have scarring in the lumen(tube) from pelvic inflammatory disease, previous tubal ligation or any other previous abdominal or pelvic surgeries that cause adhesions. Woman who smoke, have a history of multiple abortions, or have undergone fertility treatments are also at higher risk.

Ectopic pregnancies are at high risk of rupture and are considered a medical emergencies. Symptoms of an ectopic pregnancy are sharp,stabbing pain in the pelvis/abdomen, vaginal bleeding and/ or fatigue and weakness.

An ectopic pregnancy is diagnosed by a low hCG blood test, a pelvic exam and ultrasound. Treatment can be multiple things including methotrexate drug therapy or surgery. Methotrexate may be given, which allows the body to absorb the pregnancy tissue and may save the fallopian tube, depending on how far the pregnancy has developed. Laparoscopic surgery under general anesthesia may be performed. This procedure involves a surgeon using a laparoscope to remove the ectopic pregnancy and repair or remove the affected fallopian tube. If the ectopic pregnancy cannot be removed by a laparoscope procedure, then another surgical procedure called a laparotomy may be done.

When surgery is performed the specimen obtained is sent to my lab.  This is what most ectopic pregnancy specimens look like.

This is the anatomy of  the fallopian tube.

Below I have circled in black the normal anatomy of the fallopian tube which is the distal/ fimbriated end. The area circled in red is not normal. This is the site where an ectopic pregnancy ruptured. The soft tan-brown spongy tissue in this are is called chorionic villi and is considered immature placental tissue. If this tissue is found in the fallopian tube it indicates an ectopic pregnancy. Sometimes even a little embryo or fetal parts can be found next to this tissue as well.

This is a cross section of the fallopian tube. The tube is grossly dilated and filled with red-brown clotted blood. This is called a hemotosalpinx and is another feature found with ectopic pregnancies.

The pathologist needs to see a slide with chorionic villi tissue to confirm the diagnosis of ectopic pregnancy. The chances of having a successful pregnancy after an ectopic pregnancy may be lower than normal, but this will depend on why the pregnancy was ectopic and the patients medical history. If the fallopian tubes have been left in place, there is approximately a 60% chance of having a successful pregnancy in the future.