Hypoplasia of the Spleen: Review of Pathogenesis, Diagnosis, and Potential Clinical Implications.

CONTEXT: Splenic aplasia is seen when the spleen is congenitally absent, has been surgically removed, or becomes atrophic secondary to episodes of arterial/venous occlusion, which result in splenic infarction. This rare condition is caused by a heterogenous group of diseases, which may present a wide spectrum of clinical manifestations. Splenic hypoplasia is defined as reduction in splenic mass and or functions caused by incomplete splenic development or secondary parenchymal involution. Splenic infarction may be clinically silent and only discovered incidentally during abdominal exploration for other conditions. CASE REPORT: We present an unusual case of hypoplastic spleen with calcifications, which was preoperatively found during radiologic workup for gastric carcinoma. An 88-year-old woman presented with coffee-ground emesis. Her past medical history was only significant for atrial fibrillation. Esophagogastroduodenoscopy demonstrated gastric carcinoma, for which a subtotal gastrectomy was planned. Preoperative computed tomography scan showed a hypoplastic spleen with calcifications in the left upper quadrant. Symptoms of immunologic deficiency were not present. During laparotomy, an atrophied and calcified spleen was identified and left in situ. The patient made an uneventful postoperative recovery. Splenic hypoplasia is an unique entity, which may be seen in the setting of atrial fibrillation and abdominal malignancy. CONCLUSION: Splenic hypoplasia may be detected incidentally during radiologic workup or abdominal exploration. Abdominal symptoms or immunologic deficiency are not always present.

Adrenocortical Carcinoma: Complete Surgical Resection After 18 Years.

Adrenal cortical carcinoma (ACC) is a rare neoplasm often associated with an aggressive biological behavior. Complete surgical resection is the mainstay of therapy for ACC and offers the best chance for prolonged disease-free survival. We present an unusual case of a long-standing adrenal mass, well documented over a period of at least 18 years, without the development of metastatic disease, and ultimately proven to represent ACC after successful surgical resection. Physicians should be aware that ACC can present with a wide spectrum of biological behavior, from very aggressive to more indolent disease.