Suprarenal Gland Tumor Case File

Suprarenal Gland Tumor Case File

Eugene C. Toy, MD, Lawrence M. Ross, MD, PhD, Han Zhang, MD, Cristo Papasakelariou, MD, FACOG

CASE 26

An 18-year-old female presents with increasing hair growth on her face and chest, deepening of her voice, and acne over the past year. She has no history of other medical problems. On examination, she has acne, abnormal male pattern balding, and enlargement of her clitoris. The pelvic examination is normal including the ovaries. Blood tests show normal serum testosterone levels but a markedly elevated level of dihydroepiandrostenedione sulfate, an adrenal androgen.

⯈ What is the most likely diagnosis?

ANSWER TO CASE 26::

Suprarenal Gland Tumor

Summary: An 18-year-old female is seen for increasing hirsutism, deepening voice, and acne over the past year. She has no other medical problems. On examination she displays acne, hirsutism, temporal balding, and clitoromegaly. The pelvic examination, including the ovaries, is normal. The testosterone level is normal, and the level of dihydroepiandrostenedione sulfate is markedly elevated.

• Most likely diagnosis: Suprarenal (adrenal) gland tumor

CLINICAL CORRELATION

This young female has more than hirsutism, which is increased hair growth. She also has virilism, or the effects of androgens on the skin, voice, and clitoris. The hyperandrogenism seems to be of acute onset, which is consistent with an androgen-secreting tumor. The two possibilities include an ovarian tumor, usually Sertoli-Leydig cell tumor, or an adrenal tumor. Because the pelvic examination and testosterone levels are normal, an ovarian etiology is less likely. Moreover, the high level of dihydroepiandrostenedione sulfate almost establishes the suprarenal (adrenal) gland as the cause. The next step would be a CT or MRI scan of the suprarenal glands to determine the exact location of the tumor. Usually surgery is indicated. Another cause of hirsutism is polycystic ovarian syndrome, which includes hirsutism, obesity, anovulation, and irregular menses. Cushing syndrome or disease presents strong cortisol effects, such as buffalo hump, abdominal striae, easy bruising, and central obesity.

APPROACH TO:

The Suprarenal Glands

OBJECTIVES

1. Be able to describe the anatomy of the suprarenal glands

2. Be able to describe the general pattern of lymphatic drainage of the abdomen

DEFINITIONS

VIRILISM: Presence of mature male characteristics in a female or a prepubescent male

CLITOROMEGALY: Enlargement of the clitoris

DIHYDROEPIANDROSTENEDIONE (DHEA): Male steroid hormone secreted by the testis, ovary, or adrenal cortex

DISCUSSION

The paired suprarenal glands are retroperitoneal endocrine glands composed of an outer cortex that secretes corticosteroid and androgen steroid hormones and an inner medulla (derived from neural crest cells) that secretes the catecholamines, epinephrine and norepinephrine. Each gland sits on the superior pole of each kidney, enclosed within the renal fascia, and, hence, embedded in the perirenal fat. The right suprarenal gland is somewhat triangular and is closely related to the IVC, liver, and diaphragm. The left gland is shaped like a comma and related to the spleen, pancreas, stomach, and diaphragm. The suprarenal glands receive their blood supply from multiple small branches that arise from the inferior phrenic, aorta, and renal arteries. Each gland is drained by a single suprarenal vein that terminates in the IVC on the right and the renal vein on the left.

The lymphatic drainage of the abdomen is diagrammatically summarized in Figure 26-1. In general, the lymphatic drainage of abdominal organs reversely follows their arterial blood supply. Thus the lymph drainage from organs supplied by the SMA will be to the superior mesenteric nodes by way of vessels and other node groups located along the branches of the SMA. If a “final common pathway” for lymph drainage in the abdomen could be named, it would be the lumbar (aortic) lymph nodes, and lymph from these nodes drains to the cisterna chyli and thoracic duct. Lymph from the suprarenal glands drains into the lumbar lymph nodes. Figure 26-1 shows that the lymphatics from the gonads also drain to the upper lumbar nodes as the gonadal vessels arise in the upper abdomen (reflecting the site of their embryologic origin). Note also that the pectinate line in the anal canal is a watershed with regard to lymphatic drainage. The lymph from the anal canal and rectum superior to this line drains to iliac nodes; inferior to this line, lymph drains to inguinal nodes.

Figure 26-1. Lymph node drainage of the abdomen.

COMPREHENSION QUESTIONS

26.1 As a surgeon about to remove the right adrenal gland, you examine the blood supply of the right adrenal gland and observe which of the following?

A. It receives its arterial blood supply from the aorta only

B. Its central vein drains into the IVC

C. Its central vein drains into the left renal vein

D. It is in contact with the head of the pancreas

E. It lies external to the renal fascia

26.2 After removal of a large portion of the stomach in a patient who has cancer, you are now examining the lymph nodes that receive lymph from the stomach. Which of the following structures receives lymph directly from the stomach?

A. Cisterna chyli

B. Aorticorenal nodes

C. Celiac nodes

D. Superior mesenteric nodes

E. Inferior mesenteric nodes

26.3 In a patient who has testicular cancer that has metastasized (spread) to the lymph nodes, which of the following would you expect to be involved first?

A. Lumbar (aortic) nodes

B. Aorticorenal nodes

C. Inferior mesenteric nodes

D. Common iliac nodes

E. Internal iliac nodes

ANSWERS

26.1 B. The central vein of the right suprarenal gland drains into the IVC, whereas that of the right gland drains into the left renal vein.

26.2 C. The lymph of nodes located along the several arteries that supply the stomach will drain to the celiac nodes. Remember that the arteries that supply the stomach are all branches of the celiac artery.

26.3 A. Tumor cells from either gonad that metastasize through the lymphatics will metastasize to the lumbar (aortic) lymph group. Remember that the origin of the gonadal arteries is the abdominal aorta.

ANATOMY PEARL

⯈ The right suprarenal gland is closely related to the IVC, into which its vein will open.

⯈ The left suprarenal vein drains into the left renal vein.

⯈ Multiple arteries to the suprarenal glands arise from the inferior phrenic artery, aorta, and renal artery.

⯈ Lymph from the gonads drains to upper lumbar nodes.

⯈ Lymph above the pectinate line of the anal canal drains to the iliac nodes, whereas lymph below the pectinate line drains to inguinal nodes.

References

Gilroy AM, MacPherson BR, Ross LM. Atlas of Anatomy, 2nd ed. New York, NY: Thieme Medical Publishers; 2012:172−175, 236. 

Moore KL, Dalley AF, Agur AMR. Clinically Oriented Anatomy, 7th ed. Baltimore, MD: Lippincott Williams & Wilkins; 2014:294−297. 

Netter FH. Atlas of Human Anatomy, 6th ed. Philadelphia, PA: Saunders; 2014: plates 261, 308, 310−311, 319.

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