Faculty Bibliography

Learning Objective #1: Avoid premature closure when a patient presents with classic lymphoma symptoms. Our case was given a diagnosis of lymphoma by the emergency department and the hematologist oncologist consulted on the case. While the first impression was lympho-ma, there is a very rare, locally invasive neoplasm of the lymphatic vascular origin called papillary intralymphatic angioendothelioma (PILA), also referred to as Dabska tumor. Recognize that although PILA commonly presents as a slowly growing, asymptomatic (without B symptoms), violaceous patch or nodule, arising from a preexisting lymphatic or vascular anomaly that occurs typically in young adults, it may also affect the elderly and present with typical B symptoms. CASE: A 36 year-old otherwise healthy female presented with six days of acute flu-like symptoms, myalgias, persistent fever and chills associated with abdominal bloating and vomiting for three days. Physical exam was notable for temperature of 103 F, sinus tachycardia and mild tenderness at the left upper quadrant on deep palpation. Labs were notable for a normal WBC count, mildly elevated alkaline phosphatase and total bilirubin. The flu swab was negative. All infectious workup including a quantiferon-TB gold test was negative. The Chest CTshowed confluent 9 x 5 cm anterior mediastinal and 2 x 2 cm sub carinal lymphadenopathy extending to the aortopulmonary window. On abdominal CT abdomen and pelvis there was marked diffuse, bulky and confluent upper abdominal, mesenteric and retroperitoneal adenopathy. The patient underwent mediastinal video assisted thoracoscopic surgery (VATS) which showed papillary intralymphatic angioendothelioma (PILA). The patient was referred to a vascular anomalies' specialist and was prescribed Sirolimus for prophylaxis immunosuppressant therapy. IMPACT/DISCUSSION: To our knowledge, less than 40 cases of PILA have been described in literature. Presently, there are diagnostic challenges for pathologists and providers given PILA's rarity and histological complexity to distinguish it from other vascular lesions and lymphoma. We present a new and unusual case of PILAwith typical B symptoms and multifocal intra-abdominal lesions. Most case reports describe asymptomatic and external presentations. It is important to rely on final pathology prior to assuming lymphoma is the most likely diagnosis.
Conclusion(s): Premature closure is failure to consider alternative diagnoses after the initial impression is formed. This leads to diagnostic errors that can have substantial effects on our patients lives. Rare diseases are often overlooked and we need to recognize PILA as a differential diagnosis for neoplasms of the lymphatic vascular origin

Stent thrombosis is a potentially life threatening condition caused by several factors or a combination factors, such as resistance to platelet agents and type of anticoagulation used as well as stent types. We report a case of acute thrombosis and discuss potential areas of intervention with literature review.

A 43-year-old man with an unremarkable medical history presented to our hospital with 2 weeks of headaches, ataxia and confusion. CT of the head revealed a large haemorrhagic cystic lesion. A subsequent chest CT revealed a large left atrial mass. The mass was subsequently biopsied with positive immunohistochemistry staining for MDM2, FLI1 and vimentin. Real-time PCR revealed MDM2 amplification, confirming the diagnosis of intimal sarcoma. The patient underwent surgical resection and reconstruction of the atrium with subsequent discharge to short-term rehabilitation, but his symptoms continued to progress. A repeat CT of the head revealed a new cerebellar mass. He underwent a second resection, but continued to experience worsening symptoms. He was diagnosed with stage IV intimal sarcoma and referred to hospice. The patient died 5 months after initial presentation. Autopsy was performed and revealed the cause of death as pneumonia. There was no involvement of the pulmonic or aortic vessels.