Diagnosis of ovarian dysgerminoma during pregnancy

IMAGING IN MEDICINE

Diagnosis of ovarian dysgerminoma during pregnancy

José Eduardo Gauza^I; Ademir Garcia Reberti^II; Jean Carl Silva^III; Leonora Zozula Blind Pope^IV; João Cesar da Rocha dos Santos^V; Silvana Maria Quintana^VI

^IMestrado em Saúde e Meio Ambiente - Médico obstetra do Hospital Dona Helena, Joinville, SC

^IIMestre em Saúde e Meio Ambiente - Professor do Curso de Medicina da Universidade da Região de Joinville - UNIVILLE, Joinville, SC

^IIIDoutor em Ciências Médicas - Professor do Curso de Medicina da Universidade da Região de Joinville - UNIVILLE, Joinville, SC

^IVMestre em Saúde e Meio Ambiente - Professora do Curso de Medicina da Universidade da Região de Joinville - UNIVILLE-SC, Joinville, SC

^VMédico oncologista do Hospital Dona Helena, Joinville,SC

^VIDoutora em Tocoginecologia- Professora do Curso de Medicina da Universidade de São Paulo - USP-Ribeirão Preto, São Paulo, SP

Correspondence

INTRODUCTION

Dysgerminomas are tumors originating within the primordial ovarian germ cells. They are very rare, frequently malignant, and are responsible for around 1% of all germ cell tumors.^1,2 Around 20 to 25% of ovarian tumors originate in the germ cells and just 3% are malignant.³ Germ cell tumors account for around 70% of ovarian neoplasm cases, during the first decades of life, manifest malignant characteristics in 1/3 of cases and are rarely found after this period.^3,4 Dysgerminoma has a classic correlation with seminoma of the testis, having an identical histological structure.^1,2,5,6 It affects young females, usually during childhood, and patients generally respond well to chemotherapy.¹ They are generally considered to have low malignant potential, but may spread if there is invasion through metastasis or capsule, or lymph node or blood cell involvement. ^2,8,9

CASE

A white, 25-year-old female sought medical services for prenatal care.

Previous 4-years' history was healthy with no occurrences. Patient claimed no family history of neoplasm and reported menarche at 12 and regular menstrual cycles. Patient reports no previous surgeries or cervical cauterization. Pregnancies 1 Births 0; prenatal tests all normal, pregnancy progressed with no intercurrent clinical conditions. Obstetric US at 14 weeks showed an enlarged left ovary with a corpus luteum cyst. US at 19w2d normal. US at 31 weeks showed a solid mass in the left adnexa, contiguous with the uterus, measuring 179x152 mm = 1915 cm3, compatible with a myomatous node with central necrosis; fetus free from abnormalities. From this point on, the patient began to complain of mild pain focused in the left hypochondrium and radiating out to the iliac fossa on the same side and improving with non-opiate analgesics and with no other complaints. At 39 weeks the patient went into labor and elected to deliver by caesarian via mid-line infraumbilical laparotomy. The infant weighed 2700g with a 1-minute Apgar of 9 and a 5-minute Apgar of 10. On investigation of the abdominal cavity, a solid mass was found originating in the ovary, corresponding to the left adnexal volume. Other structures adjacent to the uterus were apparently unchanged macroscopically. The pathology report described Pure dysgerminoma; weighing 2855g and measuring 23.0x18.0x11.0cm; tumor limited to the ovary; capsule infiltrated, but intact; free from neoplasm on the external surface.

Mitosis rate of 5/HPF, few stroma and discrete lymphocytic infiltrate. Angiolymphatic invasion was not detected. An oncological investigation was conducted and found no signs of intra-cavity expansion. The patient was later subjected to surgery to stage the tumor, with biopsy samples taken from the left and right (L/R) iliac fossa (L/R), L/R diaphragmatic peritoneum, L/R paracolic gutter, total excision of the omentum and peritoneal lavage, in which no neoplastic tissue was found. The patient was referred for supplementary chemotherapy treatment and the final staging, according to FIGO (1985) was Stage 1 A. This study was approved by the Research Ethics Committee at the Hospital Dona Helena, Joinville, SC, Brazil, in accordance with National Health Council (Conselho Nacional de Saúde) Resolution 196/96 on research involving human beings, and written consent was obtained from the patient.

DISCUSSION

Non-epithelial ovarian tumors, originating from germ cells, are rare when compared with epithelial tumors and account for 10% of ovarian cancer cases.^1,2,4,5,10 Since this is an uncommon tumor, a diagnosis of dysgerminoma is suspected at the point that a surgeon encounters it, for the first time and predicting prognosis demands certainty that metastasis has not occurred, which will undoubtedly improve the chances of better and longer survival.^2,9,11 In the case described here, giant left-side uterine leiomyoma was diagnosed during prenatal tests and neither adnexal tumor nor ovarian neoplasm were considered. Although the 14-week obstetric US showed a corpus luteum cyst in an enlarged left ovary, to a certain extent, this corresponds to what takes place in around 70% to 80% of cases, when the US operator does not routinely measure the diameter^8,11,12. Furthermore, the fact that the US at 19 weeks and 2 days did not identify structural abnormalities, either in the fetus or the ovary, raises the question of whether or not there was a mass in the ovary at that point, since the US operator reported nothing. The specialist literature indicates that certain neoplasms may undergo geometric growth of up to around 20% of their original size in a very short period of time (1 - 2 months)^12,14,26. When the 31-week US identified a solid mass in the left adnexa, its morphological characteristics were not recorded, in terms of its structure, consistency and exact location, only its contiguity with the uterus was demonstrated. This is the reason why uterine myomatosis was suspected and when the suspicion is uterine myomatosis during pregnancy, workup is not of the same order as would be ordered if the suspicion were a malignant ovarian tumor, such as testing for tumor markers, pelvic magnetic resonance imaging and others.^2,3,4,26 Zganjer et al. suggested a hypothesis of an association between dysgerminoma and an abdominal trauma in a patient of 12 years of age involved in a traffic accident⁶. It is also known that the clinical courses of some syndromes have associations with dysgerminoma, as is the case with Cowden's syndrome¹², ataxia telangiectasia syndrome¹³, Swyer syndrome (pure gonadal dysgenesis associated with the XY 46 karyotype)^14,15, Apert syndrome (an autosomal dominant disorder)¹⁶ and Down Syndrome¹⁷. Since the patient described here was pregnant and had no phenotypical signs that would suggest relevant syndromic data, she was not asked about, or assessed for, this possibility. An association between dysgerminoma and malignant hypercalcemia^10,18,19 has been described by some authors, but in this case tests showed that the patient was within normal limits. Another peculiarity that attracts attention is the association with dysgerminoma described in some varieties of animals^20,21. This was a very large ovarian tumor, weighed at 3160 g in the operating theater, with a firm consistency, mobile, with an intact capsule and no ascites. Everything would indicate poor prognosis, but this did not turn out to be the case and the histopathological diagnosis was Stage 1 A dysgerminoma. Although the literature consulted suggests the possibility of adjuvant chemotherapy, the decision was taken not to use chemotherapy with this patient in favor of clinical follow-up because of the favorable results of the surgery conducted for tumor staging 45 days after the caesarian and because the patient's clinical status was considered stable, as it still is today, in line with reports by some authors,^22,23 which describe some unsuccessful attempts with chemotherapy, such as Ishibashi et al¹¹, who described a case of a 14 year-old refractory to a first chemotherapy session.

The objective of publicizing this case was to raise awareness of adnexal tumors which can affect females, even during an uneventful pregnancy, and which can exhibit shockingly rapid increases in volume in a very short time.

REFERENCES

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Publication Dates

• Publication in this collection
  20 Jan 2011
• Date of issue
  2010