Picture quiz: A hairy problem in gynaecology

A 19 year old UK born Nigerian nursing student, with no important medical or surgical history and regular periods, presented to her general practitioner with a history of vaginal discharge and abdominal bloating of four to five months' duration. She was not taking any medication. On abdominal palpation an ill defined swelling in the right iliac fossa, which was not tender, was found.

Her general practitioner arranged an abdominal ultrasound scan. It showed a normal uterus with a right multi-septate cyst measuring 8.2x4.9x7.8 cm. There was no free fluid in the pelvis. Her cancer antigen 125 was normal at 19 IU/ml (upper limit of reference range is 30 U/ml). The C19-9 was slightly raised at 118 IU/ml (cut off upper limit 37 IU/ml).

She was referred to the gynaecology outpatient department. After counselling she underwent a laparotomy and right ovarian cystectomy. At laparotomy, a mass was found (figs 1 and 2).

Fig 1

Questions

(1) What are the causes of abdominal swelling in women in the reproductive years?

(2) What do figs 1 and 2 show?

(3) What are the expected histological findings?

(4) Give the prognosis for this type of lesion?

Fig 2

Answers

(1) Abdominal swellings in women in the reproductive years could be caused by physiological or pathological reasons. Physiological causes include a full bladder and a fetus after 12 weeks of pregnancy. Other causes include ovarian cysts with or without ascites, and fibroids in the uterus. Other rarer causes include mesenteric cysts and appendicular mass.

(2) The pictures demonstrate a teratoma also known as a dermoid cyst.

(3) Teratomata, which are produced from totipotent cells, typically contain tissues from all three germ layers. Histology in our patient confirmed a benign cystic teratoma filled with hair and sebaceous material.

(4) Benign cystic teratomata hold a very good prognosis. Most of them can be surgically resected. Malignant transformation occurs in fewer than 2%.

Discussion

Teratoma derives from the Greek word teraton, meaning monster. These "monster tumours" arise from a totipotent germ cell (primary oocyte). The cell can give rise to all orders of cells necessary to form components of endoderm, mesoderm, and ectoderm.

Benign cystic teratomata are the most common germ cell tumour and account for 10-25% of all ovarian neoplasms. The peak age of presentation is between 20 to 40 years. The tumours are unilateral in about 80% of cases and are characteristically relatively small compared to other ovarian neoplasms. They are unilocular and enclosed within a smooth glistening serosa, which has a doughy, yielding consistency.

On section, they have a thin wall lined by epidermis from which hair shafts (fig 2) and tooth structures frequently protrude. Within the wall, it is common to find areas of calcification that prove to be bony spicules. The lumen of the cyst is filled with a thick, yellow white sebaceous secretion that is more or less admixed with matted strands of hair.

Histologically, the dominant characteristic is mature stratified squamous epithelium with underlying sebaceous glands, hair shafts, and other skin adnexal structure. Structures from other germ layers can be identified, such as cartilage, bone, and thyroid. Tumours such as these are sometimes incorporated within the wall of a pseudo mucinous cystadenoma. In about 1-2% of the dermoids, malignant transformation of the epithelium is found.

Clinical symptoms include abdominal pain, mass, and occasionally disturbances in the menstrual cycle. They must be distinguished from other causes of pelvic pain. Diagnostic modalities include ultrasound. Occasionally, diagnosis of a dermoid can be made radiographically by the shadows caused by teeth, bones, and areas of calcification.

Tumour markers

Tumour markers are substances, usually proteins, that are produced by the body in response to cancer growth or by the cancer tissue itself. Some tumour markers are specific for one type of cancer, but others are seen in several cancer types. Many of the well known markers are seen in non-cancerous conditions as well as cancer. The goal is to be able to screen for and diagnose cancer early, when it is most treatable and before it has had a chance to grow and spread.

Tumour markers (see box) are a useful tool to detect and manage mature cystic teratomata. Raised C19-9 is associated with a higher rate of bilateralism. In this young girl the other ovary appeared normal but a dermoid may appear in it in the fullness of time. Additionally, raised C19-9 and cancer antigen 125 are associated both with malignant and benign cystic teratomata, therefore interpretation of the findings must be made with consideration of the clinical condition of the patient.

Benign cystic teratomata are managed surgically, either by open or laparoscopic approach. Except in the rare instance of malignant transformation these tumours are resectable and curable. The malignant component may take the form of a squamous cell carcinoma; thyroid carcinoma; melanocarcinoma; or a sarcoma. Torsion of a dermoid on its pedicle is not uncommon and produces signs and symptoms of acute abdominal emergency; they tend to twist more than other ovarian neoplasms.

Competing interests: None declared.

       

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