Letters to the Editor
Metachronous intradural and liver metastasis from adrenocortical carcinoma
Sir, Adrenocortical carcinoma is a rare endocrine malignant tumor with an estimated incidence of 0.5-2 cases per million persons/ year, with a bimodal age distribution and overall 5 year survival from 16% to 38%.[1] Metastasis to extra-adrenal sites such as the liver, lung and bone are common due to the aggressive biology of the cancer. However, metastases to the spinal cord are a rare occurrence with an intradural location being extremely unusual. We present a case of an intradural extramedullary metastasis from a right sided adrenocortical carcinoma with two synchronous liver metastases presenting 3 years after initial diagnosis and treatment.
Case Report
A 56-year-old female patient presented with gradual onset radiating pain to both lower limbs since 4 months, with progressively increasing severity, leading to difficulty in walking. There was no history of loss of power in any limb and sphincter function was normal. She had been diagnosed with a right sided functioning adrenal mass and underwent laparoscopic adrenalectomy 2 years ago. Histopathology showed an adrenocortical carcinoma. The patient did not 666
receive any adjuvant treatment. She had been a diabetic and hypertensive since 8 years and was on treatment for the same.
At the time of the current presentation, the patient had severe radiating pain to bilateral lower limbs, aggravated on movement. Power in both lower limbs appeared normal, sensations were preserved and sphincter function was normal. Plain X-rays of the spine did not reveal any abnormality. Magnetic resonance imaging (MRI) of the lumbosacral spine showed an intradural mass lesion at L2-L3 level which was well-delineated, isointense on T1 and hypointense on T2 images, enhancing uniformly after administration of Gadolinium [Figures 1 and 2]. Radiologically, differential diagnosis of a meningioma or a schwannoma was entertained. Incidentally a lesion was noted in the visualized portion of the liver. A whole body positron emission tomography-computerized tomography (PET-CT) revealed two fluoro-deoxyglucose (FDG) avid (standardized uptake value (SUV) 35.2) lesions in segment VI of the liver with no tracer uptake noted in the spinal region [Figure 3]. Levels of 24 h urinary cortisol were raised. A CT-guided biopsy of the liver lesion revealed metastasis from adrenocortical carcinoma. In view of other extraspinal metastases, a metastatic deposit in the thecal sac (though very unusual) was also considered.
Indian Journal of Cancer | October-December 2015 | Volume 52 | Issue 4
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A decision was taken to operate on the spinal lesion in view of severe radiating pain and to prevent progression to cauda equina syndrome. The lesion was approached through a midline incision with the patient in prone position. A L2-L3 laminectomy was performed and the tumor was localized in its entirety by the use of intraoperative ultrasound (IOUS). On opening the dura, the lesion was found to be in an intradural, extramedullary location and was yellowish, firm and moderately vascular with an attachment to one of the nerve roots; and was completely excised. The symptoms of radiating pain dramatically improved post-operatively, allowing the patient to become ambulatory. Histopathology revealed the lesion to be a metastasic deposit of adrenocortical carcinoma. On immunohistochemistry, it stained positive for inhibin and negative for calretinin and chromogranin A [Figure 4].
Patient also underwent angiographic embolization of the two liver metastases subsequently. She was advised local radiation for the spine.
Discussion
Adrenocortical carcinoma is a rare endocrine malignancy with a heterogeneous presentation.[1] It has a bimodal age distribution with peaks at the first and fourth to fifth decades of life and a slight predilection for women.[1] Adrenocortical carcinomas can be functional in approximately 60% of cases with features of hypersecretion of corticosteroids, androgens, estrogen or aldosterone.[1] The treatment is by surgical excision of the tumor with optional adjuvant treatment with mitotane.[1,2,3] Although metastases
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from adrenocortical carcinoma are common, metastasis to an intradural, extramedullary location is thus far unreported, to the best of our knowledge.
Intradural metastases to the spinal cord are rare. In a report by Schick et al. of 220 spinal metastases, only 11 were intradural.[+] Perrin et al. reported a series of 10 patients of which nine patients had pain as the presenting symptom and varying degrees of functional impairment.[5] Tsai et al. presented a series of 12 patients of which eight had intradural extramedullary tumors and four had intramedullary lesions.[6] They proposed that the possible routes of spread to the spinal intradural space include arterial spread, venous backflow and dissemination through cerebrospinal fluid. All patients underwent surgery with the best outcomes in intradural extramedullary tumors and generally poor outcomes in the intramedullary group.[6,7] The location of the lesion in our case was at the L2-L3 spinal level, which is consistent with the most frequent location of intradural extramedullary metastasis in most series being the thoracolumbar spine.[5,6]
The primary diagnosis of the spinal tumor in this patient was an intradural schwannoma or meningioma (both being common intradural tumors seen in this clinical setting) considering the radiological picture, the absence of uptake on 18-FDG-PET-CT imaging[8] and the rarity of intradural metastasis from adrenocortical carcinoma. MRI is routinely performed for suspected spinal cord lesions and helps characterize the tumor and determine its exact location. The radiological picture of intradural metastases can mimic a benign nerve sheath tumor or meningioma, with the diagnosis being made only on histopathological examination.[9]
Most patients with intradural tumors are offered surgery as primary treatment. Indications for surgery include spinal
cord compression, symptomatic nerve root compression, progressive neurological deficit and the requirement of tissue diagnosis in patients with unknown primary lesions.[6,10] However, patients having complete motor deficit have a very low possibility of postoperative improvement.[7] The potential benefits of surgery have to be weighed against the risks of a major surgical procedure in these patients and careful case selection is necessary. Our patient had the disabling symptom of bilateral radiating lower limb pain suggestive of radiculopathy, which improved significantly after surgical decompression of the nerve roots. Patients considered unfit or unsuitable for surgery can be offered radiotherapy for symptom control.[2]
Conclusion
It can be concluded that metastasis from a treated adrenocortical carcinoma in a spinal intradural location is a rare possibility and a difficult diagnosis to make based on the radiological findings; but has to be kept in mind when dealing with such lesions in patients with a history of malignancy. Surgery provides marked symptomatic benefit in carefully selected cases apart from providing confirmation of diagnosis.
Acknowledgment
The authors would like to thank Dr. Asawari Patil (Consultant, Department of Pathology, Tata Memorial Hospital, Mumbai) for her valuable contribution to the case report.
Ghose J, Prabhash K1, Shetty P, Moiyadi AV Departments of Surgical Oncology, Division of Neurosurgical Services, and 1Medical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
Correspondence to:
Dr. Aliasgar V Moiyadi, E-mail: amoiyadi@actrec.gov.in
References
1. Kitano M, Patterson E, Kelebew E. Adrenal Tumors. In: Vincent T. DeVita, Samuel Hellman, Steven A. Rosenberg (2011). Cancer: Principles and Practice of Oncology, 9th Ed. Philadelphia: Lippincott-Williams and Wilkins, 2011; 1480-1488.
2. Schteingart DE, Doherty GM, Gauger PG, Giordano TJ, Hammer GD, Korobkin M, Worden FP 2005 Management of patients with adrenal cancer: Recommendations of an international consensus conference. Endocr Relat Cancer 2005; 12:667-680.
3. Wajchenberg B, Albergaria PM, Medonca B, Latronico A, Campos CP, Ferreira AV, Zerbini M, Liberman B, Carlos GG, Kirschner. Adrenocortical carcinoma: Clinical and laboratory observations. Cancer 2000;88:711-736.
4. Schick U, Marquardt G, Lorenz R. Intradural and extradural spinal metastases. Neurosurg Rev. 2001;24:1-5
5. Perrin RG, Livingston KE, Aarabi B. Intradural extramedullary spinal metastasis. A report of 10 cases. J Neurosurg. 1982;56:835-7.
6. Hong-Chieh Tsai, Hung-Lung Chen, Yung-Hsing Hsu, Wen-Hsing Hsu, Hsiu-Chuan Yen, Chih-Lung Lin. Surgical Experiences with Intradural Spinal Metastases from Systemic Neoplasms J Cancer Res Pract 2010;26:12-20.
7. Sinardet D, Chabane A, Khalil T, Seigneuret E, Sankari F, Lemaire JJ, Chazal J, Irthum B. Neurological outcome of 152 surgical patients with spinal metastasis. [Article in French]. Neurochirurgie. 2000;46:4-10.
8. Shah S, Rangarajan V, Purandare N, Luthra K, Medhi S. 18F-FDG uptakes in leptomeningeal metastases from carcinoma of the breast on a positron emission tomography/computerized tomography study. Indian J Cancer 2007;44:115-8.
9. Ng WP, Fehlings MG. Intradural metastasis mimicking nerve sheath tumor. Spine (Phila Pa 1976). 1995;20:2580-3.
10. Ahn DK, Park HS, Choi DJ, Kim KS, Kim TW, Park SY. The surgical treatment for spinal intradural extramedullary tumors. Clin Orthop Surg 2009; 1: 165-17 2.
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| DOI: 10.4103/0019-509X.178397 | |