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CASE REPORT
Year : 2013  |  Volume : 2  |  Issue : 1  |  Page : 47-48

First, the flow stopped, then the hair grew and finally, the legs gave away: A case of adrenal carcinoma with paraneoplastic myelopathy


Department of Internal Medicine, Government General Hospital, Guntur, Andhra Pradesh, India

Date of Web Publication13-Mar-2013

Correspondence Address:
A Sridhar
Post Graduate in General Medicine, FF-5, PG Hostel, Government General Hospital, Guntur - 522 007, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.108513

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  Abstract 

Adrenocortical carcinoma is a rare neoplasm with an incidence of about one case per million population. We present a rare case of an adrenal cortical tumor with paraneoplastic myelopathy and features of virilization and Cushing's disease.

Keywords: Adrenal carcinoma, Cushing′s, paraneoplastic myelopathy, rare neoplasm, virilization


How to cite this article:
Rao P M, Sridhar A. First, the flow stopped, then the hair grew and finally, the legs gave away: A case of adrenal carcinoma with paraneoplastic myelopathy. J NTR Univ Health Sci 2013;2:47-8

How to cite this URL:
Rao P M, Sridhar A. First, the flow stopped, then the hair grew and finally, the legs gave away: A case of adrenal carcinoma with paraneoplastic myelopathy. J NTR Univ Health Sci [serial online] 2013 [cited 2020 Apr 7];2:47-8. Available from: http://www.jdrntruhs.org/text.asp?2013/2/1/47/108513


  Introduction Top


Adrenocortical carcinoma (ACC) is a rare neoplasm with an incidence of about one case per million population. [1] About 60% are functional tumors that secrete hormones and present with clinical features like Cushing's syndrome due to cortisone, virilizing features due to androgens, or feminizing tumor due to estrogens. [2] We present a case of an adrenal cortical tumor with features of virilization and Cushing's disease with paraparesis.


  Case Report Top


A 25-year-old female housewife presented with paraparesis with predominantly proximal muscle weakness. She had amenorrhea since 2 yrs. Since then, she had noticed a decrease in her breast size and an increase of her facial hair. On examination, there was frontal recession of hair. She has a moon face with a beard and a mustache. Supraclavicular pad of fat was noted. Clitoromegaly was present. Her blood pressure was 170/100. Neurological examination showed upper motor type of paraparesis with motor level at T10, reflex level at T10, and sensory level at T6. A firm mass was felt in the left hypochondrium.

CT scan of abdomen and chest showed a 13 × 10 cm heterogenous mass of 37-70 HU arising from left adrenal region with nodules in the liver and lung [Figure 1] and [Figure 2]. There was paraaortic lymph node enlargement. Biochemical analysis showed normal levels of ACTH, aldosterone, and urinary vinyl mandelic acid. However there were elevated levels of dehydroepiandrostenedione, testosterone, and early morning cortisol levels. MRI of spine was normal [Figure 3]. CT scan of brain was normal.
Figure 1: CT abdomen showing adrenal mass on the left side

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Figure 2: Metastatic mass from posterior wall of left pleura

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Figure 3: MRI dorsal spine is normal

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  Discussion Top


Adrenal carcinomas account for 0.2% of all cancer deaths. [3] Most patients with adrenocortical carcinomas are diagnosed at an advanced stage of disease with large primary tumors (median tumor size at diagnosis, > 10 cm) and invasion to adjacent organs. The main clinical symptoms, such as abdominal discomfort or back pain, are related to the mass effect of a large tumor. [4] Adrenocortical carcinomas with mixed virilizing and Cushing's syndrome are typical, but pure virilizing adrenocortical carcinomas are uncommon, comprising 5-10% of the cases in most large series. [5] An adrenal carcinoma with a paraneoplastic myelopathy has never been reported before.

Our patient presented with features of hypersecretion of cortisol, androgens, and paraneoplastic myelopathy. Amenorrhea, cushingoid facies, supraclavicular pad of fat, and hypertension were due to excess cortisol in the body.

Decrease in her breast size, increased hair on her face like beard and mustache, frontal recession of hair and clitromegaly were due to hyperandrogenism.

The upper motor type of paraparesis in our patient was probably paraneoplastic as MRI of the dorsolumbar spine was normal. Review of literature shows that many patients with paraneoplastic myelopathy have a normal MRI. [6]

In addition, there were secondaries in the lung and liver. Thus, it was in a very late stage (stage 4:weiss criteria) that the patient was presented to our institution. Adrenal biopsy was associated with high mortality risk, and so the patient was referred to higher centers.


  Acknowledgement Top


Department of Radiology, Guntur Medical College.

 
  References Top

1.Bellantone R, Ferrante A, Boscherini M, Lombardi CP, Crucitti P, Crucitti F, et al. Role of reoperation in recurrence of adrenal cortical carcinoma: Results from 188 cases collected in the Italian National Registry for Adrenal Cortical Carcinoma. Surgery 1997;122:1212-8.  Back to cited text no. 1
[PUBMED]    
2.Pommier RF, Brennan MF. An eleven-year experience with adrenocortical carcinoma. Surgery 1992;112:963-71.   Back to cited text no. 2
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3.Mitra S, Roy SG, Sur PK. Adrenocortical carcinoma with skeletal metastases in a postmenopausal woman. Indian J Med Paediatr Oncol 2009;30:24-7.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.Mansmann G, Lau J, Balk E, Rothberg M, Miyachi Y, Bornstein SR. The clinically inapparent adrenal mass: Update in diagnosis and management. Endocr Rev 2004;25:309-40.  Back to cited text no. 4
[PUBMED]    
5.Cordera F, Grant C, van Heerden J, Thompson G, Young W. Androgen-secreting adrenal tumors. Surgery 2003;134:874-80.  Back to cited text no. 5
[PUBMED]    
6.Graber JJ, Nolan CP. Myelopathies in patients with cancer. Arch Neurol 2010;67:298-304.  Back to cited text no. 6
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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  In this article
   Abstract
  Introduction
  Case Report
  Discussion
  Acknowledgement
   References
   Article Figures

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