Composite pheochromocytoma-ganglioneuroma of the adrenal gland: A case report with immunohistochemical study
A 37-year-old lady presented with complaints of palpitation, headache and dyspnea for 5 months along with pain in the abdomen and off and on fever for 6 months to the Endocrine Surgery Department at Sanjay Gandhi Postgraduate Institute of Medical Science, Lucknow. She had a past history of one episode of encephalopathy as well as a hysterectomy under spinal block. There was no history of hypertensive episodes or endocrine problems in the family. USG and CT scan showed a well-defined heterogeneous left adrenal mass with solid and cystic components. MIBG Scan revealed a left adrenal mass. S. Cortisol level was 0.65 ml micro gm/dl after overnight dexamethasone suppression test (ONDST) showed normal suppression [] urinary metanephrine was elevated i.e., 2.56/24 hrs (normal <1). Rest of the general parameters were within normal limits. Left adrenalectomy was done through transperitoneal anterior abdominal approach. The operative findings showed a highly vascular nodular tumor arising from the left adrenal, lying superomedial to the left kidney. Intact capsule with dense adhesions to the retroperitoneal and left crus of the diaphragm was seen.\nLeft adrenalectomy specimen measuring 6.5 × 5.5 × 3 cm and weighing 113 g. Cut surface showed two cystic lesions each measuring 3 × 1.5 and 2 × 2 cm filled with grey brown material and rest of the surface was yellowish []. Capsule was intact.\nSections from left adrenal mass showed a tumor composed of polygonal cells arranged in well-defined nests surrounded by a delicate fibrovascular stroma (Zellballen pattern) []. These tumor cells had moderate to abundant amount of granular eosinophilic to amphophilic cytoplasm, round to oval nuclei and single prominent nucleoli. Intermixed with this tumor, are areas showing sheets of mature ganglion cells surrounded by fascicles of Schwann-like cells [Figures and ]. Areas of hemorrhage, necrosis, dense fibrocollagenous tissue and mixed inflammatory cell infiltrate were also seen within the tumor. At places, these cells showed multinucleation, intranuclear inclusions and hyaline globules which were positive for periodic acid Schiff stain (PAS). Normal adrenal gland was also seen at the periphery of the tumor. Primitive neuroblastic cells were not observed. The final histopathological diagnosis of CP-ganglioneuroma was made.\nOn immunohistochemical staining, pheochromocytes which contained neurosecretory granules were relatively strongly positive for Chromogranin A [] and Synaptophysin and were weakly positive for neuron-specific enolase (NSE) []. In gangliocytes, the NSE was strongly positive. S-100 protein in the ganglioneuroma component was positive while HMB45 and CK in the pheochromocytoma component were negative.