WeBSurg, the e-surgical reference of Laparoscopic surgery

Clinical Case

RECURRENT FEMINIZING ADRENOCORTICAL CARCINOMA

F Jamali, MD, Hôpitaux Universitaires de Strasbourg, Strasbourg, France

1. Description

We report on the history and imaging studies of a patient previously operated on for an adrenocorticocarcinoma, who presented with a recurrent adrenal tumor.

2. Keywords

Cancer, adrenal, recurrence

3. Patient

Female, 71-year-old

4. Past medical history

Medical: hypertension, stable angina
Surgical: cholecystectomy, appendectomy

5. History of present illness

The present illness dates back 4 years ago when the patient presented with clinical signs of hyperestrogenism, namely breast enlargement and excessive vaginal discharge.
Physical exam was unrevealing.

6. Laboratory findings

Screening laboratory values are normal except plasma estradiol: 178 pg/mL (N<30)CA-50, CA-125, CA 19-9, CA 15.3 and CEA: N
Hormonal workup was otherwise negative (metanephrine, normetanephrine) ruling out a pheochromocytoma.

7. CT scan I

Right adrenal lesion measuring 2.5 cm in diameter

Figure 7.a

Figure 7.b

Figure 7.c

Figure 7.d

8. Treatment I

- laparoscopy: gross appearance consistent with invasive carcinoma;
- conversion to open with wide resection.

9. Pathology

Secreting adrenocortical carcinoma completely resected. The lesion had a high mitotic index, abnormal mitotic figures with capsular invasion into the peri-adrenal fat tissue.

10. Follow-up

The estradiol level normalizes postoperatively. The metastatic workup is negative and the patient receives no adjuvant therapy. 4 years later, the patient complains again of mastodyna and excessive vaginal discharge. Hormonal workup reveals elevation of estradiol and decreased FSH and LH.

11. CT scan II

The rest of the imaging workup is negative.

Figure 11.a

Figure 11.b

Figure 11.c

12. Treatment II

Exploratory laparotomy with resection of the aorto-caval lesion and aorto-caval lymph node dissection.
The rest of the abdominal exploration is negative.

13. Staging of adrenocortical carcinoma

Stage I: Lesion <5 cm in diameter. No lymph nodes involvement or distant metastasis
Stage II: Lesion >5 cm in diameter. No lymph nodes involvement or distant metastasis
Stage III: Lymph node involvement without distant metastases
Stage IV: Distant metastases

14. Discussion

Feminizing adrenal tumors are exceptionally rare. They are malignant in >90% of the cases and represent 1-2% of adrenocortical carcinomas. Adrenocortical carcinoma tends to occur in the first decade as well as the fourth and fifth decades of life, and is slightly more common in women. The tumors are classified as functional or nonfunctional, depending on tumor production of corticosteroid, androgen, estrogen, or mineralocorticoid. Most patients (>70 %) present with large masses and with stage IV disease. Common sites of metastases are liver, lung and bone. Abdominal computerized tomography and magnetic resonance imaging are used in the evaluation of intra-abdominal disease. The most effective treatment for adrenocortical carcinoma and the only potentially curative one is complete resection. Early stage and curative resection are the two clinical factors that are of prognostic significance for long-term survival and are associated with a 5 year survival in the range of 30-45%. Mitotane is the chemotherapeutic agent most often used to treat adrenocortical carcinoma. Its efficacy in prolonging survival is limited, but may be enhanced by monitoring of serum levels and their maintenance at elevated values. Even for patients who undergo complete resection, recurrent and metastatic disease are extremely common. The only effective treatment for recurrent disease is reoperation aiming at complete resection.

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