Nonfunctioning adrenocortical carcinoma.SM Lele, BV Mittal, IM Vora, HS Kulkarni
Dept of Pathology, Seth GS Medical College, Parel, Bombay, Maharashtra.
The rarity of adrenocortical carcinoma prompted us to report a case who came with a history of swelling in the left flank associated with pain, weakness and loss of appetite. Ultrasonography revealed a left retroperitoneal mass which was removed by radical surgery along with the left kidney and spleen. On histopathological examination, a diagnosis of adrenocortical carcinoma was made. (Hough criteria score 2.97). The cells of the tumor were arranged in closely packed columns and cords supported by fibrovascular stroma. There was no evidence of metastasis.
Keywords: Adrenal Rest Tumor, surgery,ultrasonography,Case Report, Human, Male, Middle Age, Retroperitoneal Neoplasms, surgery,ultrasonography,
Adrenal cortical carcinomas (ACC) are very rare tumours, occurring in two patients per million per year according to the statistics in several large cancer registries,. Their relative incidence among malignant tumours is 0.2%. The rarity of this lesion prompted us to report this case.
A 54-year-old male patient came with complaints of swelling in the left flank since 1½ years. This was accompanied by pain, weakness and loss of appetite. On examination, he had pallor and oedema feet. His blood pressure was normal. There was a lump in the left hypochondrium, 22 cm in diameter. The mass was ballotable. Liver was not palpable. There was a left sided pleural effusion.
Ultrasonography of abdomen revealed a left retroperitoneal mass, separate from the left kidney and spleen. Liver scan showed no evidence of metastasis.
Serum creatinine (1.2mg%), BUN (16 mg%), fasting blood sugar (112 mg%) and electrolytes were within normal limits. On exploratory laparotomy the mass was found to be in the position of the left adrenal. Left kidney, spleen, and tail of pancreas were normal. Radical surgery was carried out to remove the lump, left kidney and spleen.
The mass removed weighed 1060 gms; measured 20 x 14 x 10 cm; was encapsulated and bosselated without any breach in the capsule (See Fig. 1). The left kidney and spleen were not adherent to the mass and did not show any metastasis on gross examination.
Microscopic examination revealed a cellular tumour composed of cells arranged in closely packed columns and cords supported by a delicate fibrovascular stroma. The cells were round, oval and at places polygonal. The nuclei were hyperchromatic and pleomorphic. Occasional large multinucleate giant cells were seen scattered amidst the tumour cells. Fair number of mitotic figures were seen. One section showed capsular invasion (See Figure:2]), however, there was extracapsular infiltration by the tumour. The peri-adrenal fat, spleen, left kidney and omental fat showed no-evidence of metastasis. There were no venous emboli in the hilar vessels.
Based on these findings, the diagnosis of adrenocortical carcinoma was made.
Instances of cortical hyperplasia and adenoma are found commonly at post-mortern examination of asymptomatic individuals. However, most of the non-functioning adrenocortical lesions seen as surgical specimens are carcinomas,. These non-functional tumours are therefore difficult to diagnose and the infrequency with which these tumours are seen, make it difficult for an individual physician to gain experience with this tumour or to suspect its origin in the adrenal gland.
ACC may be classified as non-functioning and functioning according to the clinical syndromes with which they are associated (adrenogenital syndrome, Cushing's or Corin's syndrome). Malignant process, at times, may be associated with absence of some of the enzymes required for cortisol synthesis resulting in the production and release of steroid precursors like dehyroepiandrosterone and 11 deoxycortisol. The presence of the latter is a consequence of a deficiency of 11 ? hydroxylase enzyme which is a characteristic feature of ACC. These cases, with absence of steroid hormones are non-functioning. Urinary metabolites of the precursors can be used as tumour markers for the diagnosis and follow-up of above-mentioned non-functioning ACC.
Grossly, the non-functioning tumours are similar to the functioning tumours but are generally larger. Some authors have found no differences between the two types, on histology, while others have found a cord like and alveolar pattern in cases without a hormonal syndrome. Lewinsky et al have described more compact cells in non-functioning tumours. Our case showed a predominantly cord like pattern.
Several excellent series comparing benign and malignant adrenal cortical neoplasms have been reported,,,,. However, a lot of controversy exists between the workers as to the strict criteria to distinguish between the benign and malignant lesions. Short of evidence of metastasis, however, no single criterion is diagnostic. More recently, Hough and associates have described a mathematical scoring system involving histologic and non- histologic criteria, which when considered together are more diagnostic than a single feature considered alone. Histologic criteria include diffuse pattern of growth, vascular and capsular invasion, tumour cell necrosis, broad fibrous bands, mitotic index and pleomorphism. Each of these is scored. A score of 2.91 ? 0.9 is considered are tumour mass, urinary 17 ketost-eroids, no increase in 17 hydroxy steroids after 50 ?gm of ACTH, presence of functional activity and weight loss. In our case, urinary ketosteroids were not estimated pre-operatively and the diagnosis of ACC was only made on histopathology. Besides biochemical investigations, excretory urography, ultrasonograghy, computerised tomography and arteriography are other diagnostic tools extremely useful for a pre-operative diagnosis, especially in non-functioning tumors.
The two most common sites of distant metastasis are the lungs (53%) and the liver (44%). Metastases to the bones and brain are unusual. There was no metastasis seen in our case.
Early surgical resection is the key to the cure. Aggressive surgical resection of metastasis may be beneficial, Combination of surgical treatment and chemotherapy has been advocated for recurrent disease. The role of radiation alone has had little documentation in literature. The median survival is two years. However, there is variation in survival according to the histologic grades. Grade III cases survive for one month while Grade II survived for 51 months after surgical treatment. Radical surgery was carried out in our case.
We are grateful to the Dean, Seth GS Medical College for allowing us to publish this case report.
[Figure - 1], [Figure - 2]