Introduction
Recent literature shows an increased interest in cancer complicating pregnancy. This is a result of the realisation that oncological treatment modalities, including surgery and chemotherapy, can be applied after the first gestational trimester without hampering the fetus1,2.
Evidence from western countries shows that mainly breast cancer and hematological malignancies are diagnosed during pregnancy3. Gynaecological cancers also significantly contribute to the problem. Cancer of the cervix is the second most common cancer among women worldwide and the most common gynaecological cancer in the developing world4.
Incidence rates of cancer complicating pregnancy therefore vary around the world. Especially with this perspective in mind, guidelines for the treatment of gynaecological cancer were recently proposed5. In contrast, sarcomas are uncommon and increase with age. Apart from bone sarcomas, we are not aware of other sarcomas complicating pregnancy. Here, we describe a fatal case of a high-grade endometrial stromal sarcoma (ESS) diagnosed at a gestational age of 19 weeks.
Case presentation
A 28-year-old Caucasian woman consulted her gynaecologist with pain in the right fossa at a gestational age of 15 weeks. Her medical history was straightforward. She smoked 10 cigarettes per day for more than 10 years. Sonographic examination suggested an appendicular plastron and was interpreted as an ovarian mass. Subsequently, a laparoscopy was performed in a district hospital.
Due to the pregnancy and the adhesions the view was incomplete (the uterus and ovaries could not clearly be identified) but peritoneal spread of malignant plaques was evident. Microscopic examination of the peritoneal lesions showed a solid, fat-infiltrating mass, composed of cancerous cells with storiform growth pattern.
Cancer cells have a spindle form containing a moderate quantity of eosinofilic cytoplasm and a polymorph vesicular nucleus, sometimes containing a prominent nucleolus. More than 10 mitotic figures per 10 high-power fields were present, including abnormal mitotic figures. This morphology corresponds to a high grade sarcoma. Immunohistochemistry was performed and the tumour cells revealed the following immunophenotype: desmin (-), alpha SMA (+++), CK7 (+), CK20 (-), CD117/C-Kit (-), S100 (-), CD34 (-), C125 (-), EMA (-), CD10 (diffuse +++), calretinine (-), CK 5.6 (-), MDM 2 (-), ER (-), PR (-). The positive staining for CD10 and alpha-smooth muscle actin (alpha-SMA) in the absence of desmin expression may be indicative for a sarcoma of endometrial stromal origin.
Chromosome preparations from the tumour specimen were obtained using standard primary culture procedures. For diagnostic purposes the karyotype was determined:
66-71<3N>,XXX,+X,-1,der(2)t(1;2)(p35;q37)),-7,+11,-13,-14,der(14;15)(q10;q10),-15,-16,+17,der(18)t(7;18)(q11;q23),+20,+21,+21 [cp17]. Hence, the tumour karyotype was not specific for any known translocation-related or other sarcomas.<p></p>
In order to exclude the possibility of KIT-immunonegative gastrointestinal stromal tumour, mutational analyses were performed using a combination of polymerase chain reaction (PCR) amplification, denaturing high-performance liquid chromatography (D-HPLC) pre-screening, and bi-directional sequencing, as described previously6.
Tumour specimen showed wild-type genotype for exons 9, 11, 13, 17 of the KIT or exons 12, 14 and 18 of PDGFRA genes. Thus, the mutational analysis was not indicative for any particular sarcoma. Therefore, the final diagnosis was most suggestive for high-grade ESS.
Subsequently, she was transferred to our hospital. Magnetic resonance imaging showed diffuse peritoneal and omental tumoural implants, spreading along the visceral surfaces of the small bowel and large bowel, without a definable primary mass (Figure 1). Also a moderate amount of ascites was present. There were no signs of hepatic and lymph node metastasis. Computer tomography of the lungs excluded metastasis.
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| Figure 1. Magnetic resonance imaging findings of diffuse peritoneal involvement by a poorly differentiated sarcoma. Sagittal T2-weighted turbo spin-echo magnetic resonance image (repetition time msec/echo time msec = 8440/136) shows diffuse sheetlike and nodular thickening of the peritoneal surfaces (arrows). Note also a moderate amount of ascites (asterisk). Bladder (B). |
We discussed the diagnosis of a high-grade ESS with transperitoneal spread, but without distant metastasis, with the patient and her husband. Psychological support was provided. We explained that the situation was life threatening for both the mother and fetus. Given the young age of the patient and expected limited response to chemotherapy, we opted for a maximal surgical effort during cytoreductive surgery.
If this had been a case of a significant cytoreduction, we would have considered anthracyclin based chemotherapy, even in the presence of an ongoing pregnancy. We agreed that if the maternal situation seemed prospectless, termination of pregnancy should be performed. In which case, hysterotomy would appear to be a better solutio