Saudi Journal of Kidney Diseases and Transplantation

: 2011  |  Volume : 22  |  Issue : 1  |  Page : 153--155

Giant adrenal cortical carcinoma

Onkar Singh, Shilpi Singh Gupta 
 Department of Surgery, M.G.M. Medical College & M.Y. Hospital, Indore, India

Correspondence Address:
Onkar Singh
Department of Surgery, M.G.M. Medical College & M.Y. Hospital, Indore

How to cite this article:
Singh O, Gupta SS. Giant adrenal cortical carcinoma.Saudi J Kidney Dis Transpl 2011;22:153-155

How to cite this URL:
Singh O, Gupta SS. Giant adrenal cortical carcinoma. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2021 Jun 15 ];22:153-155
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Full Text

To the Editor,

Adrenal cortical carcinoma (ACC) is a rare tumor that accounts for only 0.02% of all can­cers. [1] It has a bimodal age distribution, with peaks in the first and fifth decades of life. About 60% of the patients present with symp­toms of excessive hormone production. How­ever, ACC is usually non-functional when it occurs in adults. Most patients with non-func­tional ACC present with stage IV disease with large abdominal masses. [2],[3] Most ACCs are large but weigh in the range of 0.1-1 kg. [4] The largest ACC reported was 29 cm × 19 cm × 10 cm in size, weighing 4.7 kg. [5] We herewith re­port a case of a huge non-functional ACC of the left adrenal gland that measured 22 cm × 18 cm × 15 cm and weighed 2.8 kg.

A 47-year-old male presented with complaints of dull, vague abdominal pain localized to the left upper quadrant for the last 2.5 months. This pain was not associated with vomiting, fever or any other complaint. The patient was hemodynamically stable. Abdominal examina­tion revealed a large lump of around 20 cm × 15 cm located in the left hypochondrium. It was smooth, firm-to-hard in consistency and was not moving with respiration. The patient was admitted and further work-up was carried out. Ultrasonography of the abdomen was per­formed, which showed a solid mass at the upper pole of the left kidney. The chest radio­graph did not reveal any abnormality. A com­puted tomography (CT) scan showed the organ of origin of the lump as left adrenal gland, which was involving the upper pole of the left kidney [Figure 1]. The liver and the opposite adrenal were normal. Functioning of both kid­neys was normal on intravenous urography performed along with CT scan. Levels of ad­renal hormones were normal. The patient was subjected to left-sided adrenalectomy. The left kidney also needed to be removed as the tumor involved its upper pole. Histopathological and immunohistochemical examinations of the excised specimen confirmed the diagnosis of non-functional ACC [Figure 2] and [Figure 3].{Figure 1}{Figure 2}{Figure 3}

ACC is a rare, aggressive tumor that affects only one to two persons per million population. [1] It represents 0.02% of all cancers and has a poor prognosis, causing 0.2% of all cancer deaths. [6] ACC can be functional or non-func­tional, depending on the production of cortico­steroid, androgen, estrogen or mineralocorticoids by the tumor. Approximately 60% of the pa­tients present with symptoms related to exce­ssive hormone secretion, others being non-functional. Non-functional ACCs are slightly more common in women (58.6%) than they are in men (41.1%) and have a bimodal age distri­bution, with peaks in the first and fifth decades of life. In children, ACC is usually functional while in older adults, these are diagnosed com­monly because of their mass effect, local inva­sion or by metastases. Most patients with non­functional ACC present with large masses in stage IV disease. [2],[3] ACCs usually weigh bet­ween 100 g and 1 kg, and sizes larger than this are very rare. [4] By searching the Pubmed/Med­line and the internet via various search engines, we found that the largest ACC reported so far was 29 cm × 19 cm ×10 cm in size and 4700 g in weight. [5] Thus, to the best of our knowledge, the present case represents the second-largest ACC, weighing 2.8 kg.

All patients with suspected ACC should be evaluated for signs and symptoms of hormonal syndromes, including Cushing's syndrome, Conn syndrome, feminization and hypokale­mia. ACCs are usually not biopsied. The size of the adrenal tumor on CT or magnetic res­onance imaging (MRI) is the best indicator of malignancy. [6] The diagnosis of ACC is con­firmed after its removal. Grossly, areas of he­morrhage and necrosis, breached capsule and tumor weight and, histologically, presence of invasion and increased mitotic activity suggest malignancy. [6] Staging of ACC consists of de­termining the size of the primary tumor, the degree of local invasion and spread to regio­nal lymph nodes or distant sites. [1] The staging system, recommended by the WHO and based on the TNM classification is widely used. [7] Stages I and II include localized tumors ≤ 5 cm and >5 cm, respectively. Locally invasive tu­mors or those with regional lymph node me­tastases are classified as Stage III. Stage IV consists of tumors invading the adjacent or­gans or tumors with distant metastases. [6] Proper staging should include a CT scan of the abdo­men, but it is often unable to predict the organ of origin in large tumors. [8] MRI adds specifi­city to the findings of CT scan. [9] Selective angiography and adrenal venography are help­ful in identifying smaller lesions and distingui­shing these from tumors of the upper pole of the kidney. [8] The lung and liver are frequently affected by metastases and, therefore, CT of the chest and abdomen is mandatory. [6]

The most effective treatment for ACC is complete resection, which is the only poten­tially curative treatment option. [3] But, majority (60%) are diagnosed in Stages III or IV, being too late for curative resection. [2] However, re­section can be performed even in the case of invasion of the renal vein or inferior vena cava. [10] The 5-year survival rate after successful surgery is 50-60%. Even after complete re­section, recurrent and metastatic disease is very common. [3] Palliation by radiotherapy and radiofrequency ablation may be offered to those who are not surgical candidates. [10] However, radiotherapy is the treatment of choice for most metastases; metastases at selected sites and local recurrences can sometimes be palliated surgically. [6],[11] A combination chemotherapy re­gimen consisting of cisplatin, doxorubicin and etoposide along with mitotane is widely used to eliminate microscopic residual disease after surgery or to make an unresectable tumor ame­nable to surgery. [10] Mitotane, an inhibitor of steroid synthesis, is the most often used chemo­therapeutic agent and is shown to increase sur­vival. However, prolonged treatment is often limited by gastrointestinal and neurologic toxi­city. [3],[10] Endocrine cell toxin streptozocine has also been included in some treatment protocols. [10]

In conclusion, huge adrenaocortical carci­noma is very rare and this case has been re­ported because of the rarity of this tumor being so gigantic, and, to best of our knowledge, this may be the second-largest ACC reported so far.


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