Wael Elsaify

Lesson of the month 2: Catecholamine-induced cardiomyopathy – pitfalls in diagnosis and medical management

Cardiomyopathy as the initial presentation of phaeochromocytoma (PCA) is uncommon. Diagnostic work-up and perioperative management may be challenging within this context. We report three cases of PCA presenting with cardiomyopathy to illustrate the pitfalls in diagnosis and management. None of the patients had typical adrenergic symptoms and all three were established on beta-blockers prior to diagnosis. Their fractionated plasma catecholamine levels were elevated and the diagnosis of PCA was confirmed with various imaging modalities and post adrenalectomy. Interpretation of fractionated catecholamine levels in the context of established cardiomyopathy is difficult as cardiac failure of any aetiology generates an adrenergic response. Hence screening all patients with idiopathic cardiomyopathy is likely to generate a high false-positive rate. However, a high index of suspicion should prompt further diagnostic work-up in patients with idiopathic cardiomyopathy for occult PCAs. Peer-reviewed guidelines are required to guide the investigation and management of suspected catecholamine-induced cardiomyopathy.

Management of adrenocortical carcinoma

A 46-year-old woman presented with a 2-week history of a painless swelling in the left upper abdomen. Physical examination revealed a visible and palpable mass in the left upper abdominal quadrant. An abdominal ultrasound scan demonstrated a 14-cm well-defined mass, possibly left adrenal in origin. Plasma catecholamines, renin, aldosterone and cortisol were within normal ranges. Subsequent computed tomography (CT) imaging showed a well-defined, heterogenous 15.6 × 11.7 × 16.8 cm retroperitoneal mass, displacing the lower pole of the left kidney but not arising directly from it (Fig. 1). The left adrenal gland could not be identified separately. Following multidisciplinary team discussion, a laparotomy and left adrenalectomy was deemed the most appropriate management. The mass was excised completely intact and analyzed in the laboratory. The tumour dimensions were 160 × 120 × 143 mm with a weight of 1935 grams. There was a recognizable adrenal gland present at one edge of the specimen measuring 35 × 15 mm. The cut surface of the tumour had a nodular, solid and fleshy appearance with large areas of necrosis and haemorrhage present (Fig. 2). Histologically, the tumour was composed almost entirely of solid sheets of eosinophilic epithelial cells with foci of coagulative necrosis (Fig. 3). The nuclei were moderately pleomorphic with foci of marked pleomorphism. Mitotic activity was frequent (21 mitotic figures per 50 high-power fields) with occasional abnormal mitotic figures noted. The tumour was encapsulated and confined to the adrenal gland by the adrenal capsule. These findings were those of an oncocytic adrenocortical carcinoma, completely excised, with staging pT2 NX R0. Adrenal masses are among the most frequent tumours in humans, with the vast majority being benign. Adrenocortical carcinoma (ACC) is a rare, very aggressive malignancy affecting only 1–2 million people per year. ACC has a bimodal distribution, in which there is a relatively high incidence in children less than 5 years of age and in adults in their fourth and fifth decades of life. ACC often presents with symptoms of hormonal excess or a palpable mass. In approximately 60% of cases, the presentation of

Lobular carcinoma of the breast: presentation, histopathological features and management of rectal metastasis.

hand, cluster of differentiation antigen 68; CD 68 (PGM1; 1:40 dilution, DBS) was expressed in OGCs whereas tumor cells were negative for it (Fig. 3). The characteristic histology and immunohistochemistry findings established a final diagnosis of invasive duct carcinoma breast (Bloom Richardson Grade II) with OGCs was given. Breast carcinoma with OGCs is a rare entity categorized separately under the WHO classification of invasive ductal carcinoma, not otherwise specified. This tumor is characterized by the presence of OGCs admixed with malignant epithelial cells. Most likely, the OGCs derive from macrophages, possibly in response to hypervascular microenvironments induced by breast cancer, with secretion of cytokines that might play a role in progression of cancer. OGCs must be differentiated from the tumor giant cells in the setting of a malignant neoplasm. The tumor giant cells are bizarre in appearance and exhibit pleomorphism and nuclear atypia, whereas the OGCs have numerous centrally located round to elongated, regular nuclei without significant atypia. Immunohistochemistry further aids in differentiating the two as CD 68 is expressed in OGCs whereas the tumor giant cells are negative for it. The distinctive cytomorphologic features of this unusual tumor make FNA a reliable modality in making its early and confident diagnosis, thereby assisting the clinician with better management of the case.