Renal Angiomyolipoma Causing Inferior Vena Cava Thrombus in a Young Girl With Tuberous Sclerosis.

Angiomyolipomas (AML), also known as hamartomas, are benign mesenchymal tumors of the kidneys which consist of vascular tissue, smooth muscles, and adipose tissue, with a higher prevalence in females than males. AML may be associated with tuberous sclerosis, and the growth of the mass may present as hematuria or flank pain. We present a case of a 14-year-old female patient who had a known case of tuberous sclerosis since early childhood. She has a history of numerous bilateral renal masses radiographically consistent with AML. A special and unique entity of our case is the age of presentation which is 14 years and the presence of TSC. In contrast to our case, which was invading the right renal vein and IVC at a young age, AML is well known for its benign nature. According to a recent literature review, the youngest patient reported was 16 years old. Typically, non-complicated AMLs less than 4 cm in size are managed by annual radiological imaging which is preferably a CT scan, while larger AMLs of more than 4 cm that present with perinephric hemorrhages or intralesional aneurysms are treated by partial nephrectomy or selective angioembolization. A radical nephrectomy and IVC thrombectomy are typically necessary due to the risks that the IVC thrombus carries as well as the AML itself and its unpredictable behavior. In cases like ours with the extension into the renal vein and IVC, the surgical approach is similar to the venous invasion of renal cell carcinomas.

Upper aero-digestive tract lymphomas presenting as polypoidal/pedunculated lesions: case report and review of literature.

AIM: To describe a case of diffuse large B cell lymphoma (DLBCL) presenting as a pedunculated/polypoidal mass in upper aero-digestive tract and to review pertinent literature. METHODS: Using Pubmed advanced search, case reports and literature were reviewed for this condition. RESULTS: A 16 year old female presented with history of shortness of breath when lying down, voice change, progressing odynophagia and feeling of throat lump. Direct visualization showed flower like pedunculated mass, attached to the left lateral oropharyngeal wall. Excision of this mass showed DLBCL and no disease anywhere. She received four cycles of Rituximab + CHOP and is disease-free for 4 years. Only 21 cases of pedunculated upper aero-digestive tract lymphomas have been reported in literature. Median age 53 years (16-90 years), males 80 %, females 20 %, localized disease (65 %). Common sites were endobronchial polypoid lesion (7 cases), nasal polyps (7), nasopharyngeal polyps (2), nose and paranasal sinus (1), laryngeal polyp (2) and one each as gum, esophagus and oropharyngeal (current case) polyp. Pathology showed aggressive lymphoma in 94 %. CONCLUSION: DLBCL as an extranodal pedunculated lesion in the oropharynx is very rare. Careful pathological evaluation is essential for these lesions for proper diagnosis.