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Christopher B-Lynch


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Acute Abdomen Secondary To Rare Yolk Sac Tumour

Arch Gynecol Obstet  DOI 10.1007/s00404-008-0796-z
Hooman Soleymani Majd • Sally Ann Turner •
Kiyoshi Choji • Khin Lwin • Christopher B-Lynch
Received: 15 July 2008 / Accepted: 1 September 2008
© Springer-Verlag 2008
Abstract A 36-year-old woman presented unwell, with abdominal pain. A pelvic mass was found and being investigated but when she deteriorated and became peritonitic. An emergency laparotomy was performed and she required a pelvic clearance for a Stage IIB ovarian tumour, later confirmed as a yolk sac tumour. Accurate staging and tumourreductive surgery strongly affects the prognosis of yolk sac tumours. Thus ability to perform a meticulous surgical clearance may well positively impact on the outcome for these young patients. This case describes the challenges that generalists will be faced with when highly suspicious cancer patients present as an emergency.
Keywords Female • Yolk sac tumour • Cancer •Centralisation
Case report
A 36-year-old woman with Ehlers–Danlos syndrome, presented to A&E on Christmas day with a 12-h history of
abdominal pain and associated nausea, vomiting and bloating. She reported that over the last 4 months she had been experiencing worsening episodes of abdominal pain. There was no weight loss, urinary or bowel symptoms. Her past medical history included irritable bowel syndrome, mild asthma and a previous appendicectomy. There was no signiWcant gynaecological history. She was having regular menstrual cycles and had two previous vaginal deliveries. Her usual medications included NSAID’s, Tramadol, Buscopan and Amitryptilline. She was a youth worker, who had smoked five cigarettes per day for the past 15 years.

On examination she was pyrexial, with a temperature of 38.1°. Her pulse rate was 100 bpm and she had a normal blood pressure. There was abdominal distension and generalised tenderness to palpation but no guarding or rigidity. A mass arising from the pelvis was palpated. Her pregnancy test was negative and all the blood results were normal; white cell count was 8.8 (4–11 £ 109 L¡1) but the CRP was raised to 64 (0–10 mg L¡1).

An abdominal ultrasound demonstrated ascites and a 13 cm heterogeneous mass arising from the right side of the pelvis, as well as nodularity of the omentum. A computerised tomography was then performed, conWrming that the mass was probably ovarian in nature. It was separate from the uterus, enhanced heterogeneously and contained solid and cystic areas within it (Fig. 1). Omental nodules in keeping with metastatic deposits and ascites were again demonstrated

(Fig. 2). The other intra-abdominal organs were normal, as were the lungs, mediastinum and bones. There
was no signiWcant lymphadenopathy. At this stage routine tumour markers were sent oV. Her case was discussed at the regional oncology multi-disciplinary team (MDT) meeting and an ultrasound guided
biopsy of an omental nodule was suggested, which was performed immediately. She was stable but remained in hospital for a few days, whilst awaiting the histology results and re-discussion of her case at the regional MDT meeting, in order to decide how and where to best manage her case further.

Despite being on intravenous antibiotics the patient’s pyrexia persisted and her CRP continued to rise. On the
6th day after initially presenting, her condition deteriorated and she became acutely peritonitic and tachycardic, necessitating an urgent laparotomy. The tumour marker results became available, just prior to surgery. They showed a raised CA125 of 167 (0–35 U mL¡1), an AFP of 9640 (0–10 kU L¡1) and a LDH of 460 (85–285 U L¡1), suggestive of ovarian malignancy.

Because malignancy was highly probable, a consultant colleague who had been oncologically trained was called in to perform the surgery. A midline laparotomy was performed and a clinical diagnosis of Stage IIB ovarian cancer was made. She was found to have a large pelvic mass arising from the right ovarian adnexa, enlarged paraaortic lymph nodes and omental caking. The tumour had ruptured, leaking fluid into the peritoneal cavity. A pelvic clearance including a total abdominal hysterectomy, bilateral salpingo-oophorectomy, lymphadenectomy, omentectomy,and peritoneal washings were performed in an attempt to achieve optimal debulking and minimise spread of the tumour.

Histology confirmed a yolk sac tumour, containing areas of tumour necrosis and haemorrhage. There were scattered eosinophilic globules and Duval–Schiller bodies throughout the specimen. Perivascular sinus formation was also demonstrated and the tumour stained positively with alphafoetal protein. The ultrasound guided omental node biopsy performed prior to surgery also contained tumour cells and Duval–Schiller bodies, consistent with metastases. The patient has since been referred to the germ cell tumour team in Charing Cross Hospital, London.

Discussion

Yolk sac or endodermal sinus tumours are rare ovarian malignancies. Their incidence increases sharply around
puberty and decreases in older age groups [1]. The mean age is 17.6 years. The most frequent presentation is that of abdominal pain with a large pelvic mass. At the time of presentation the average size of the tumour is usually 14 cm and tumours are frequently unilateral [2]. Histologically, yolk sac tumours exhibit a range of
growth patterns which may mimic several other primary and secondary ovarian tumours. Reticular, endodermal
sinus, polyvesicular-vitelline, hepatoid, and glandular architectures represent the main morphologic patterns. Secondary features include Schiller–Duval bodies and intraand extracellular hyaline globules [3]. Both serologic and immunohistochemical alpha-fetoprotein expression characterise this tumour [4].

In terms of management there may be some argument for conservative surgery in younger women who have not
yet completed their family, if tumours are diagnosed early or are small and unilateral. Some sources would say that yolk sac tumours may be managed by removing the aVected ovary only and then referring the patient to a cancer centre for further chemotherapy, as yolk sac tumours are usually quite chemo-sensitive.

However, it has also been well established that staging and tumour-reductive surgery strongly aVect the prognosis of the disease and that tumour-reductive surgery is advisable when ascites is minimal [5]. If the disease is extensive, then a pelvic clearance would be indicated.

Olaitan and McCormack recently made a good argument for centralisation of services for the management of ovarian cancer, expressing the view that these cases should be referred to a tertiary centre for surgery and adjuvant therapy due to the highly aggressive nature of the tumour and the likelihood of recurrence [6]. However, this argument is questionable and may not always be possible.

Fig. 1 CT Scan Showing Large Ovarian Mass

Fig. 2 Right Omental Nodule Which Was Biopsied

In the UK, most tertiary oncology centres have quite strict acceptance criteria. They will usually only accept
patients where a diagnosis could not be made at the district level hospital or else they would expect a histological diagnosis prior to the patient being referred. These strict criteria are in place to streamline the oncology service, reduce inappropriate referrals and prevent a backlog of patients still waiting for a diagnosis before deWnitive treatment can be offered and planned. And with the new changes being introduced in the training of obstetrics and gynaecology in the UK, new consultants are becoming less familiar and relatively deskilled at gynae oncology surgery. The new structured training programmes are rather focusing their efforts on improving the core theoretical knowledge of trainees so that they are able to identify cancer patients, organise investigations and refer them appropriately to cancer centres.

Whilst our patient’s case had been discussed at the regional oncology MDT within 4 days of presentation, the
centre requested histology before the patient could be formally referred, during which time she deteriorated. This leads onto the question of what happens when complications arise in these patients, as there is usually no time to refer them. Leaving generalists to manage complications arising in patients awaiting elective surgery or patients who present acutely before a diagnosis is conWrmed. Thus when highly suspicious cancer patient presents as an emergency to a district level hospital, generalists are the one’s faced with the challenge of how to improve the outcomes and prevent morbidity for their patients.

In this case, a fully trained oncologist who was a member of staff at the local hospital, was called in to perform the surgery. However, other patients in other units may not have this resource. Thus patient’s will be managed by general gynaecologists as realistically there is no facility to be able to “call in” a specialist gynae oncologist to perform surgery at short notice for emergencies.

Even though the trend these days is generally in favour of centralisation, there remains much debate about whether or not to centralise oncology services [7]. Ultimately the debate will be based on hard outcomes pertaining to survival and progression-free survival of cancer patients.

If all oncology cases are going to be electively referred to tertiary centres then it will become increasingly difficult for generalists to maintain their skills in surgery and indeed staging when faced with the unexpected. Surely the new training scheme needs to accommodate by having contingencies for these types of situations but in the interim the challenge for generalists will continue. How are they to deal with these situations effectively and to what level and how far do they need to improve and indeed maintain their surgical skills to do so?

References
1. Moller H, Evans H (2003) Epidemiology of gonadal germ cell cancer in males and females. APMIS 111(1):43–48. doi:10.1034/j.1600-0463.2003.11101071.x
2. Devouassoux-Shisheboran M, Schammel DP, Tavassoli FA (1999) Ovarian hepatoid yolk sac tumours: morphological, immunohistochemical and ultrastructural features. Histopathology 34(5):462–469. doi:10.1046/j.1365-2559.1999.00650.x
3. Narita T, Moriyama Y, Ito Y (1988) Endodermal sinus (yolk sac) tumour of the liver. A case report and review of the literature.  J Pathol 155(1):41–47. doi:10.1002/path.1711550108
4. Rabban JT, Zaloudek C (2006) Ovarian yolk sac tumors. Pathol Case Rev 11(1):50–57.doi:10.1097/01.pcr.0000196575.32729.d6
5. Nawa A, Obata N, Kikkawa F, Kawai M, Nagasaka T, Goto S et al (2001) Prognostic factors of patients with yolk sac tumors of the ovary. Am J Obstet Gynecol 184(6):1182–1188. doi:10.1067/mob.2001.113323
6. Olaitan A, McCormack M (2007) Centralization of services for the management of ovarian cancer: arguments for. BJOG 114(10):1188–1190. doi:10.1111/j.1471-0528.2007.01460.x
7. Crawford SM, Brunskill PJ (2007) Centralisation of services for the management of ovarian cancer: arguments against. BJOG 114(10):1183–1185. doi:10.1111/j.1471-0528.2007.01461.x

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