Adrenocortical carcinoma is a erratic tumor afflicting only 1-2 persons per one million populations. It usually occurs in adults and the median age at diagnosis is 44 years. The cause is unknown. Although potentially curable at early stages, only 30% of these malignancies are confined to the adrenal gland at the calculate of diagnosis. Because these tumors tend to pocket house found years with they began growing, they be inflicted with the opportunity to invade nearby organs, spread to distant organs (metastasize) and cause numerous changes in the late because of the surplus hormones they produce.
Tumors of the adrenal gland are classified as any functioning or nonfunctioning:
• A functioning adrenocortical tumor ended produces fastidious hormones. It may trigger symptoms.
• A nonfunctioning adrenocortical tumor doesn't ooze hormones. It may not cause symptoms early on.
The symptoms associated with functioning adrenocortical tumors vary. They depend on which hormones are overproduced and on the patient's age.
Our patient Mr. IQS is a young male aged 37 yrs, is known case of recurrent non functioning (nonsecretory) cruel right adrenocortical carcinoma with adhesion to liver and IVC. He had undergone debulking surgery of right adrenal mass in approximately sickbay on 21/2/2011. Per-operative consequence was generous 12x10x15 cm tumor in suprarenal province relating sub hepatic IVC, second-rate go up of right lobe of the liver. Tumor was believer to right kidney, right renal vein and IVC. Approximately 75% of tumor was indifferent leaving behind a cuff of tumor adherent to IVC and liver.
Post operatively he was evaluated by chief medical oncologists at various hospitals and advised for T. Temozolamide 250 mg for 5 days each month for 6 months. Even this was ongoing after 15/5/2011. Meanwhile he had reappearance of disease with large right adrenal mass. He was evaluated at several hospitals and advised to undergo calming chemotherapy as they found it as an inoperable disease.
Patient came to Dharamshila Hospital on 31/7/2011. His contemporary MRI dated 28/6/2011 and whole body PET CT Scan dated 6/7/2011 showed large heterogeneous mass in right suprarenal region measuring 17.1x17.1x14 cm. Mass was displacing the right lobe of liver with adhesion to its inferior surface. Right kidney was also displaced with loss of solid smooth between them. IVC was also displaced antero medially with mass appearing adherent to the IVC just below the caudate lobe. No intra luminal big defects in IVC. There was no free fluid or other metastasis. There was no distant or nodal metastasis on PET CT.
Slide reviewed in our hospital also incorrigible the diagnosis of adrenocortical tumor with necrosis. Case was discussed in tumor penetrate and plotted for surgery if resectable followed by adjuvant treatment. With limited refund other modalities like chemotherapy and radiotherapy, other than surgery, patient's relatives and patient were given the options of surgery versus chemotherapy with mitotane. They resolute to go so far to be with surgery flush with of explained distinguished risk.
Patient at that time underwent exploratory laparotomy with resection of large right adrenal tumor with right nephrectomy owing to under general anesthesia on 13/7/2011. Perop finding - large necrotic tumor measuring approximately 25x20 cms arising early right adrenal area approaching liver up with adhesion to second part of duodenum, IVC, right kidney and hepatic flexure of colon. It was also infiltrating the retroperitoneum. It was separated from duodenum and colon. While separating from inferior surface of liver, the tumor bombard was breached. Approximately 2.5 kg of necrotic tumor mass was removed. Remaining capsule of the tumor started flow of blood profusely. It was proscribed by abdominal packing. IVC and vein were exposed. Right renal vessels isolated, ligated and cut. The left over tumor with outstanding right adrenal gland and right kidney everywhere resected leaving behind a small part of part of capsule adherent to IVC. Hemostasis may possibly not be achieved despite of all events as it was spread bleeding. Hence abdominal packing was done with some hemostasis. Abdomen was clogged with no. 32 drain in situ.
Intra operatively patient expected 29 units of packed red cells (PRC), 18 units of fresh frozen plasma (FFP) and 6 units of unsystematic donor platelets (RDP) along with other encouraging measures. Gradually patient's condition improved in ICU. He was weaned off ventilator on second post operative day. Removal of abdominal packs and clot migration with peritoneal lavage done under GA on 16/07/2011.
He received several blood units and other blood components in the post operative cycle (43 PRC, 67 FFP, 10 RDP and 7 single donor platelets (SDP) in total). Gradually over the period his condition improved with good supportive care. He was shifted to HDU on 20/07/2011. Then he was shifted to ward on 22/07/2011. Later he was discharged on 29/07/2011 in a satisfactory condition. Now patient is doing water supply on follow up visit. He is planned for adjuvant radiotherapy to tumor bed and IV chemotherapy along with mitotane.
Three consultant surgical oncologists, Dr. Niranjan Naik, Dr. Mudit Agrawal and Dr. Vinod Tikku performed the surgery. Good support of anaesthesia team ( Headed by HOD of Anaesthesia, Dr. Neha Agrawal), blood layer facility (Headed by HOD of Pathology, Dr. R. Dawar) and full OT and ICU team contributed to the successful outcome of the patient.
Adrenal carcinoma may be curable if treated at an early stage. Radical surgical excision is the treatment of scale for cancers which have not spread to other organs. This ruins the only method by which long-term cure may be achieved. Approximately 40% of all patients who undergo a radical resection of this cancer will be alive 5 years later.
In a retrospective, nonrandomized comparison of patients with first recurrence of adrenocortical cancer, 18 patients were treated with chemotherapy (primarily mitotane) and 15 patients were treated with surgical resection plus similar chemotherapy. Surgical resection of recurrent adrenocortical cancer was regularly extensive, with morbidity in 20% of patients and no mortality. Mitotane therapy was ineffective at scheming tumor growth. Median survival from the time of diagnosis for all patients was only 23 months and no patient was cured. Disease-free interval greater than 12 months was associated with prolonged survival, but it only occurred in six patients (18%), with a similar frequency in in cooperation treatment groups. Surgical resection of recurrent disease was associated with prolonged survival from the time of first recurrence. The potential benefit of this resection was evident in the 5 patients (33%) who were able to live greater than 5 years from the time of first recurrence with improvement in symptoms and signs of hypercortisolism. Although no patient with recurrent adrenal cancer could be cured, resection of recurrent disease was associated with a affront continuation of survival and good palliation of Cushing's syndrome.
