Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!

Background Mixed cortico-medullary adrenal carcinoma (MCMAC) is an extremely rare entity with scarce literature on its cytomorphology. Case presentation A 2-year-old girl presented with abdominal pain for 3 days and a past history of fever with significant weight loss. On examination, a non-tender left hypochondrial firm mass and an enlarged left supraclavicular node were found. Twenty-four-hour urinary levels of VMA were marginally high. Contrast-enhanced computed tomography of the abdomen showed a suprarenal heterogeneous mass encasing major vessels. Aspiration cytology of both mass and node showed similar features comprising a predominant population of singly scattered large cells with moderate cytoplasm, eccentric nucleus and prominent nucleolus in a necrotic background. Tumour cells expressed Synaptophysin and Melan-A. In view of increasing respiratory distress, debulking surgery was performed, and histopathology of the specimen revealed the presence of both malignant medullary and cortical components supported by immunohistochemistry making a final diagnosis of MCMAC. The patient succumbed to death in the postoperative period. The cytology slides were reviewed and were seen to show a dual cell population. Conclusion Coexistent malignant cortical and medullary tumour of the adrenal gland is the first case reported in the paediatric age group in the literature with only three previous case reports in adults.

Rare Case of Huge Adrenocortical Carcinoma in Surprisingly Asymptomatic Patient

Introduction: Adrenal Carcinoma (AC) is a rare endocrine malignancy of the adrenal glands. It can affect up to 3–10% of the human population. It is common in children as secondary malignancies or as a manifestation of a tumor syndrome. Typically patients with AC start to become symptomatic after extensive tumor development- 10–13 cm. The presentation of AC includes signs of hormone excess. The most common hormone in excess is cortisol. Thus individuals with AC typically present with symptoms of hypercortisolism or Cushing’s Syndrome. In chronic cases the high concentration of glucocorticoids can start stimulating aldosterone receptors causing hypertension and hypokalemia. Furthermore, patients with AC also have symptoms of tumor growth, such as abdominal pain, flank pain, and early satiety. Currently prognostic factors for AC are undefined. AC generally has a poor prognosis as a majority of patients are diagnosed during advanced stage of disease (stage IV). Furthermore, it was found that patients with stage IV disease had a survival of less than one year. Currently the only curative treatment is complete tumor resection. Medical management with chemotherapy can be utilized in cases of extensive metastasis and poor prognosis. A common chemotherapeutic agent is mitotane, which is a steroidogenesis inhibitor. Case Presentation:Patient is a 19 year old female, who initially presented to her primary care provider’s (PCP) office for an annual wellness visit during August 2020. She was subsequently discovered to have elevated blood pressure. At this time she was not symptomatic. She was advised on lifestyle modifications. She was also stopped on her birth control medication. Furthermore, she later presented to her PCP with continued elevated blood pressure, still not symptomatic. In an outpatient cardiology office, she presented with blood pressures in the 200s/100s range and a new murmur. She was sent to the ED for evaluation (11/2020). On her presentation to the ED, she still did not have any symptoms. She also denied any fatigue or unintentional weight changes. Her only concern was mild edema in the lower legs after standing for extended periods of time. Her medical history was insignificant. Her family history was significant for her paternal essential HTN diagnosed in the early 20s. Laboratory evaluation revealed unremarkable electrolyte levels and CBC. Furthermore, her TSH level was also normal. A chest ray revealed multiple pulmonary nodules ranging from 3–5 mm. A renal ultrasound revealed a large mass in the RUQ (16.9x9.6x12 cm). She was also found to have mildly elevated cortisol levels at 22.9, suppressed ACTH levels, and normal levels of aldosterone and metanephrines. Subsequent CT imaging and biopsy revealed adrenal carcinoma with extensive metastasis to the lungs and liver. Patient was referred to Oncology service to start Chemotherapy.

Two giant retroperitoneal schwannomas mimicking adrenal malignancy – a case report

Schwannomas are benign tumors in 95% of cases and very rarely occur in the retroperitoneum. We report the cases of a 35-year-old man with abdominal discomfort and a 50-year-old asymptomatic woman with large retroperitoneal masses. Both underwent multivisceral surgery to exclude an adrenal carcinoma, and the pathologic diagnosis showed schwannomas in both cases. Despite morphological imaging, it was not possible to get a clear diagnosis preoperatively.