Eur J Cardiothorac Surg 2001;19:516-518
© 2001 Elsevier Science NL
Metastatic granulosa cell tumour of the diaphragm 15 years after the primary neoplasm
R.J. Williamsa,
H.M. Kamelb,
A.N. Jilaihawia,
D. Prakasha
a Department of Thoracic Surgery, Hairmyres Hospital, East Kilbride, Glasgow, G75 8RG Scotland, UK
b Department of Histopathology, Hairmyres Hospital, East Kilbride, Glasgow, G75 8RG Scotland, UK
Received 26 June 2000;
received in revised form 23 December 2000;
accepted 25 January 2001.
Corresponding author. Tel.: +44-1355-220-292; fax: +44-1355-234-064
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Abstract
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We present the case of a female patient who complained of dyspnoea and was found to have a pleural effusion. A tumour involving the right diaphragm was seen on CT and after excision this was shown to be a recurrent granulosa cell tumour, 15 years after the original ovarian lesion had been treated by oophrectomy and radiotherapy. The case and literature relating to such a rare intra-thoracic metastatic tumour is discussed.
Key Words: Pleural effusion malignant • Granulosa cell tumour • Diaphragm
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1. Case report
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A 71 year old female presented with a 2 month history of lethargy, anorexia and slight dyspnoea. A chest radiograph revealed a right pleural effusion and lower lobe consolidation; she was therefore referred to the thoracic surgical unit. Fifteen years earlier, she had undergone a left oophrectomy for an ovarian tumour. There was no other abdominal involvement at the time of operation and pathological examination of the ovary showed the specimen to be a granulosa cell tumour; she received subsequent radiotherapy to the pelvis.
On admission all routine blood tests were normal, CT scanning (Fig. 1)
confirmed the pleural effusion and revealed a mass on the right diaphragm. There was no evidence of intra-abdominal tumour or ascites on CT of the abdomen. Initially the patient underwent video assisted thoracoscopy, where 2 l of blood stained fluid was aspirated from the right pleural cavity and soft necrotic plaques seen on the right diaphragm. Biopsies were taken and histological examination showed thickened pleural tissue with active inflammation and haemosiderin deposition with lymphoid aggregation. Histological examination of the biopsies suggested primary carcinoid of the lung with some atypical features.
Thoracotomy was subsequently performed, a soft haemorrhagic friable mass appearing to originate from the dome of the right diaphragm and measuring 10x5 cm. As the lesion had been previously, biopsies frozen sections were not performed and the lesion was excised together with an ellipse of diaphragm and a small area of adherent lung. The lower lobe was partially compressed but otherwise appearances were normal. Primary closure of the diaphragm was achieved without tension, the lung re-expanded and the thoracotomy closed.
The specimen appeared to be a completely excised tumour originating from the diaphragm and invading the lower lobe. Histological examination showed the lesion to be a haemorrhagic granulosa cell tumour (Fig. 2)
with areas of necrosis extensively infiltrating the diaphragm and directly invading the adjacent lung. The diagnosis was subsequently confirmed with positive immunohistochemistry for alpha-inhibin and melon-A. The 5x3 cm specimen of diaphragm showed tumour to be confined to the pleural surface with the peritoneal surface unaffected. The adjacent lung tissue also showed infiltration by similar tumour cells. Sections were compared to the original ovarian specimen excised 15 years previously; the two showed similar morphology with the ovarian lesion also strongly positive for alpha-inhibin.
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Fig. 2. High power micrograph (x400) showing characteristic appearance of granulosa cell tumour: High nuclear to cytoplasmic ratio, uniform tumour cells, nuclei with prominent grooves giving a ‘coffee bean’ appearance and inconspicuous nucleoli. A characteristic Call Exner body is seen (arrow), where cells can be seen to be arranged around small spaces containing nuclear fragments.
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After a good recovery from her surgery the patient is under the review of the oncologists who, in view of the absence of evidence for recurrence at any other site and the slow natural history of this disease, have decided to give no further treatment but follow her clinically and monitor oestradiol, CA125 and CEA levels, which are all currently normal. The patient remains well 9 months after surgery.
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2. Discussion
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Granulosa cell tumours (GCT) represent 3–10% of ovarian neoplasms [1,2], around half of such tumours are thought to be malignant and long-term survival is common [1]. The recurrence rate, after initial surgical treatment, has been estimated at 18.6% and the mean time to presentation with metastases 6 years [2], although much later is not uncommon.
Most GCT metastasis arise within the abdominal cavity, via peritoneal seedlings [1–3]. Metastases to the lung have been reported, and in the series reported by Evans and co-workers three of 22 metastases were to the lung as compared to 18 which were intra abdominal or pelvic with one at an unknown site. Only one case of disease metastasising to the diaphragm has been reported in the past [4] and in this patient disease was also found below the diaphragm at the right crus. The authors, therefore, postulated that the disease had invaded through the diaphragm from the abdomen into the chest.
The case we report above is, to our knowledge, the first of GCT recurring to the thoracic diaphragm without separate involvement of the lung or abdomen. GCT although an unusual ovarian tumour is known for its slow course and recurrence many years after its original treatment, in this case 15 years, but in the past metastases have presented 37 years after initial tumour excision [5]. Primary granulosa cell tumours are associated with excess oestrogen production in 75% of cases and rarely can be androgenic [6]. Because the diagnosis of secondary GCT was not suspected, hormone levels and tumour markers were not measured prior to surgery. The literature on the levels of these markers in metastatic lesions of this type is sparse and unhelpful.
The case we have reported above illustrates that an initial presentation which might suggest advanced malignant diseases if investigated fully until a definite histological diagnoses is made, may reveal an unusual tumour which is amenable to simple surgical measures with a subsequent good prognoses. Certainly a past history of ovarian tumour resection warrants further thought and measurement of appropriate tumour markers when such a patient presents.
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References
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Dinnerstein A.J., O'Leary J.A. Granulosa-theca cell tumours. Obstet Gynecol 1968;31(5):654-658.[Medline]
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Evans A.T., Gaffey T.A., Malkasian G.D., Annegers J.F. Clinicopathalogic review of 118 granulosa and 82 theca cell tumours. Obstet Gynecol 1980;55(2):231-237.[Abstract/Free Full Text]
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Pankratz E., Boyes D.A., White G.W., Galliford B.W., Fairey R.N., Benedet J.L. Granulosa cell tumours. Obstet Gynecol 1978;52(6):718-723.[Abstract/Free Full Text]
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Cooper G.J., Bull A.D., Kennedy A.H., Wilkinson G.A.L. Metastasis from a granulosa cell tumour of the ovary presenting in the chest but arising below the diaphragm. Respir Med 1991;85:327-329.[Medline]
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Hines J.F., Khalifa M.A., Moore L.J., Fine K.P., Lage J.M., Barnes W.A. Recurrent granulosa cell tumor of the ovary 37 years after initial diagnoses: a case report and review of the literature. Gynecol Oncol 1996;60:484-488.[Medline]
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Peckham M., Pinedo H., Veronesi V. Oxford textbook of Oncology. Oxford: Oxford University Press, 1995:1310-1313.
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Abstract
1. Case report
