Pathological Case of the Month
Nandita Kakkar, MD; R. K. Vasishta, MD, FRCPath; Amit Lamba, MD; Amita Trehan, MD; R. K. Marwaha, MD
A
23-DAY-OLD full-term, vaginally deliv- ered boy weighing 3.2 kg at birth had ab- dominal distension and excessive weight gain. At age 23 days he weighed 4.6 kg and had cushingoid features, edema, hir- sutism, and stria on the abdomen. Blood pressure was elevated (170/110 mm Hg). On examination a mass was palpable in the right hypochondriac and lumbar region. The liver was enlarged and firm. Ultrasonography re- vealed a well-defined mass in the right suprarenal re- gion with a heterogeneous echotexture. Both kidneys were visualized and showed medullary calcinosis. Serum cor- tisol levels were normal. At autopsy a right suprarenal tu- mor measuring 6 × 4×3 cm and weighing 125 g was re- moved. Gross and microscopic appearances are shown in Figures 1, 2, 3, and 4.
From the Departments of Histopathology (Drs Kakkar, Vasishta, and Lamba) and Pediatrics (Drs Trehan and Marwaha), Postgraduate Institute of Medical Education and Research, Chandigarh, India.
PM16535
Diagnosis and Discussion
Congenital Adrenocortical Carcinoma
Figure 1. Well-encapsulated tumor measuring 6× 4× 3 cm pushing the right kidney downward. The photograph has been taken from the posterior aspect.
Figure 2. Tumor cells arranged in nests with intervening blood vessels (hematoxylin-eosin, original magnification × 140).
Figure 3. Capsular invasion by the tumor (hematoxylin-eosin, original magnification × 140).
Figure 4. Metastatic deposits in the liver (hematoxylin-eosin, original magnification × 280).
T his child presented with a rare entity: congeni- tal adrenocortical carcinoma with microscopic metastasis to the liver. The tumor revealed 6 of 9 histologic criteria laid out by Medeiros and Weiss,1 which are found to be associated with adrenal cortical neoplasms that metastasized or recurred. These are cap- sular invasion, necrosis, sinusoidal invasion, mitosis (>5/50 high-power field), diffuse architecture (>33% of tumor), and eosinophilic tumor cell cytoplasm (>75% of tumor). Above all, the sine qua non of malignant tu- mors (metastasis to the liver) was also seen.
Adrenocortical carcinoma in children is rare2-4 and comprises only 0.2% of all childhood malignant tu- mors.5 They comprise 6% of childhood malignant adre- nal tumors, most being neuroblastomas.6 Congenital ad- renocortical carcinomas are even more rare, and few cases have been published in the world literature.7-9 In con- trast to adults, most of these tumors in children are hor- monally active, and perhaps early detection is the rea- son for their so-called better survival. Clinically, they manifest by virilization, Cushing syndrome, and occa- sionally with aldosteronism and feminization (in de- creasing order of frequency). A mixture of syndromes is the most common mode of presentation. There is greater incidence of functional adrenal tumors in females than males for unexplained reasons. The occurrence of Cush- ing syndrome resulting from an adrenal cortex tumor in infants in association with hemihypertrophy and uri- nary tract anomalies as well as various other anomalies suggests oncogenic factors occurring during embryonic development.2,9,10 Carcinomas outnumber adenomas in childhood adenocortical tumors.
Most children with adrenal tumors have signs of an- drogen excess,3,11 including acne, deepening of voice, mus- cular appearance, pubic hair, rapid statural growth, and en- larged penis or clitoris. The differential diagnoses in a virilized boy are quite limited and include late-onset con- genital adrenal hyperplasia, Leydig cell tumor of the tes- tis, and true isosexual precocity. Virilization associated with features of hypercortisolism or feminization occurs twice as frequently as “pure” virilization.12 Virilizing tumors tend to be small (~50 g). Endogenous Cushing syndrome in chil- dren younger than age 10 years is usually due to an adre- nal tumor,13 whereas in older children, hyperplasia may
exceed tumors in frequency.14 Cushing syndrome with- out virilization is rarely observed. The classic features of Cushing syndrome include obesity, muscle wasting, plethora, round facies, striae, short stature, and hyperten- sion. Linear growth may be normal in children with asso- ciated virilization. Truncal obesity may be observed in older children, but infants tend to demonstrate generalized obe- sity. Primary aldosteronism is rare in children and is usu- ally due to adrenocortical hyperplasia, but few cases are re- ported in the literature in which primary aldosteronism is due to an adrenocortical tumor.15,16 Pure feminizing tu- mors are rarely encountered in children. Only 7 cases of feminizing adenomas in males have been recorded.17 About 5% of childhood adrenocortical tumors produce no clini- cal evidence of endocrine dysfunction. This does not mean that they are incapable of steroidogenesis but only that they do not produce an excess of active hormones. Because these tumors do not produce symptoms, they are extremely large at the time of diagnosis and have poor prognosis.
Accepted for publication May 28, 1999.
Reprints: Nandita Kakkar, MD, Department of Histo- pathology, PGIMER, Chandigarh-160012, India.
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