Renal Angiomyolipoma (AML) is a benign
neoplasm common in women of childbearing age. Literature shows many cases in which tumour mass was complicated with intra tumoral haemorrhage during pregnancy demanding emergency intervention. Here is a case in which elective excision of tumour mass was done in a 20 weeks pregnant female preserving kidney and without any harmful effect on foetus.
A 25 yr. old Oman female, Primigravida (20 wk.), presented with progressive increase in size of lump in left hypochondrium noticed during antenatal checkup. There was no history of fever, haematuria or other lower urinary tract symptoms. General examination revealed normal vitals with no pallor or icterus. Per abdominal examination showed gravid uterus consistent with 20-wk. pregnancy. An indiscreet mass was felt in epigastrium, left hypochondrium and left lumbar region.
Magnetic Resonance Imaging revealed a 19 x 16 x 21 cm irregular mass occupying left upper abdomen with extension across the mid line seeming to involve mid pole of left kidney. Multiple vessels were seen throughout the mass. It was abutting aorta and Inferior Venacava and displacing them and other abdominal organs to right (Figs. 1 and 2).
Considering the size and pregnant status of patient, exploration was planned under presence of an obstetrician and paediatrician to manage any mishap to foetus during the course of surgery. 11th rib approach was taken and retroperitoneally mass was dissected. Large number of dilated veins were ligated. Tumour was abutting hilum of kidney. It was separate from aorta and Inferior Venacava. A plane was created between mass and renal hilum and complete excision of mass was done preserving kidney and safeguarding major vessels. Foetus was monitored throughout the course of surgery and there was no undesirable incident and patient continued her pregnancy.
Histopathology showed fat, vascular and smooth muscle elements consistent with diagnosis of AML. Immunohistochemical profile showed presence of oestrogen and progesterone receptors in tumour tissue (Fig. 3).
Fig. 1 : STIR axial showing the large mass and its
relationship with other major organs.
Fig. 2 : Non-contrast angiography showing the large
feeding vessel and other intratumoral vessels.
Fig. 3 : Microphotograph showing positivity for
progesterone receptors on immuno-histochemical staining.
AML is a benign, non-capsulated, slowly progressing renal neoplasm composed of fat, vascular and smooth muscle elements. Overall incidence is 0.3-3%.
Two types are described as follows :
Isolated AML - sporadic, solitary, accounts for 80% of tumours, more common in women.
AML associated with Tuberous Sclerosis-larger, bilateral, multiple and accounts for 20% of tumours.
Most AML are asymptomatic but around 40% are symptomatic and present as abdominal mass, haematuria, flank pain, or shock due to spontaneous intratumoral haemorrhage. With advances in cross-sectional imaging, diagnosis of AML can be established without surgery. It is more common in women of childbearing age. Pregnancy favours the development of Angiomyolipomas and during pregnancy Angiomyolipomas tend to rapidly increase in size.1
In a study done by L'Hostis et al, Immunohistochemical profile of renal AML showed positivity of oestrogen and progesterone receptors in more than 25% of cases.2
In our case, major risk was of abrupt termination of pregnancy, which would have turned to be big emotional set back to patient and relatives. Also, due to pregnant status, we were unable to subject patient to investigations properly. Loss of renal tissue in already compromised pregnant patient was another hurdle in our plan of management. However, with improved surgical and anaesthetic skills, good post operative monitoring we were able to excise the tumour without any morbidity to the patient and allowing pregnancy to continue till term.
Renal AML is a benign, slow growing neoplasm common in women of childbearing age. Rapid growth in size may be due to intratumoral haemorrhage or pregnancy, consistent with presence of oestrogen and progesterone receptors.
In a unique case as ours, first trimester was uneventful and subsequently patient was symptomatic due to rapid growth in tumour probably due to pregnancy induced changes in hormonal milieu of body. Pregnancy also increases risk of haemorrhage in tumour. Clinical experience of surgeon is required to decide for proper management before any dreadful complication to patient or foetus occurs.
Further research is needed to prove that AML growth could be affected by hormonal factors and that hormonal therapy could prevent the growth of AML. Thus, probably surgical removal could be avoided in some cases.3
I am grateful to Mr. Vaibhav Dicholkar (Librarian, Lilavati Hospital) for his continuous help in preparing this report.
- Alberto Cozzoli, Alessandro Teppa, Gina Gregorini. Spontaneous Renal hemorrhage occurring during Pregnancy. J Nephrol 2003; 16 : 595.
- L'Hostis, Helene, Deminiere, et al. Renal Angiomyolipoma; A Clinicopathologic, Immunohistochemical, and Follow up study of 46 cases. American J Surgical Pathology 1999; 23 (9) : 1011.
- Helen Logginidou, Xiang AO, Irma Russo, Elizabeth Petri Henske. Frequent estrogen and progesterone receptor immunoreactivity in renal angiomyolipomas from women with pulmonary Lymphangioleiomyomatosis. Chest 2000; 117 : 25-30.
PROFILE AND PREVALENCE OF ASPIRIN RESISTANCE IN INDIAN PATIENTS WITH CORONARY ARTERY DISEASE
This study shows that aspirin resistance and aspirin semi responsiveness do ocur in the Indian patients and there are no reliable clinical predictors for this condition. The diagnosis therefore relies primarily on laboratory tests.
In the controls (n=35) with 10 µm of ADP the mean platelet aggregation was found to be 54.33 ± 16.81% and with 0.5 mg/ml of AA it was found to be 56.00 ± 21.93%.
We also foresee further advancements in the diagnostic tests for aspirin resistance like PFA-100 and estimation of 17-hydroxy TXA, which are user-friendly. Once these are commercially available the true picture of aspirin resistance may come to light.
Indian Heart J, 2005; 57 : 658-661.