Non Hodgkin's Lymphoma is a predominantly nodal disease. Hepatic involvement secondary to systemic disease is common-however, a primary hepatic location of non Hodgkin lymphoma is rare and only constitutes 0.01% of all cases. This is a case of a 51 year old female who presented with an abdominal swelling measuring 10 cm. Imaging showed a large mass in the right lobe of the liver and biopsy revealed non Hodgkin lymphoma-diffuse large B cell lymphoma. Patient was treated with 6 cycles of RCHOP chemotherapy and patient followed up regularly for 12 months with resolution of mass on imaging and resolution of symptoms. This case highlights a rare case of primary non hodgkin's lymphoma of the liver and how it was successfully treated with chemotherapy alone. Case Presentation: This is a case of a 51 year old female who presented with an abdominal swelling for 2 months associated with decreased appetite and weight loss. On examination, there was a tender, well-defined, firm to hard mass measuring 10 cm in the epigastric region. Ultrsound of the Abdomen showed hepatomegaly with large ill-defined hyperechoic lesion predominantly in right lobe of liver and moderate ascites with internal echoes. CECT showed a 13.8 x 9 cm mass in left lobe of liver without distant metastasis and was suspected to be a primary hepatic neoplasm. Liver biopsy showed non hodgkin lymphoma-diffuse large B cell lymphoma-germinal center subtype/ high grade B cell lymphoma with MYC and BCL6 rearrangement. Immunohistochemistry showed diffuse membrane positivity for CD20 and CD10, diffuse nuclear positivity for BCL6, CMYC nuclear positive in >40% cells, and Ki67-75%. Patient was treated with pre phase chemotherapy with rituximab and steroids. She was treated with RCHOP chemotherapy and Peg filgrastim. 6 cycles of RCHOP were given in total, PET CT done post chemotherapy in comparison with PET CT done pre chemotherapy showed interval resolution in metabolic activity of the large hypodense lesion in liver and interval resolution in size and metabolic activity of the mediastinal lymph nodes. Patient followed up for 6 months and is asymptomatic on follow up.

Juvenile Xanthogranulomas (JXGs) are rare, benign lesions that belong to the large group of non-Langerhans cell histiocytosis, often affecting infants and younger children. JXG usually presents with 1 or more erythematous or yellowish nodules that are most commonly located on the head or neck. On the contrary, we present a rare case of juvenile xanthogranuloma which presented as a large swelling in the gluteal region-a location that has been rarely described in literature. The baby underwent excision of gluteal swelling. Histopathology demonstrated a lymphohistiocytic lesion characterized by proliferation of mononuclear medium-sized histiocytes along with admixed multinucleated giant cells, variably lipidized xanthomatous and Touton-type giant cells. Immunohistochemistry showed immunopositivity for CD68 (Diffuse) and CD4 (Diffuse). Cutaneous JXGs are usually self-limiting and do not require treatment, unless large and causing functional impairment as seen in the case described. Systemic cases may even warrant chemotherapy. Case Report History-A 1-month-old child was brought with insidious onset right gluteal swelling over one month. The swelling was non-tender, with reddish discoloration of the overlying skin. Clinical Examination-The child was alert, active, and hemodynamically stable. Systemic examination was normal. Local examination showed a 4x2 cm swelling in the left gluteal region, which was non-tender and soft to firm in consistency. It was fixed to the underlying structures. The overlying skin was pinchable and seen to be mildly erythematous. No ulceration or discharge was present. No sacral abnormality was noted. The anal opening was normal. Investigations-Baseline investigations reported elevated AFP levels, and serial AFP levels reported a decreasing trend. Ultrasound showed a well-defined hypo-echoic lesion measuring 3.1 cm x 1.9 cm noted in the medial aspect of the left gluteal region at the level of the ischium in close relation with the perianal region, showing internal vascularity-possibly sacrococcygeal teratoma. MRI Pelvis subsequently showed a large lobulated altered signal intensity solid lesion showing predominantly peripheral diffusion restriction and heterogenous post-contrast enhancement arising adjacent to the base of the coccyx with a large exophytic component in the left gluteal region. CT Thorax showed no obvious metastases.

Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterised by oculocutaneous albinism, a bleeding diathesis and platelet storage deficiency. The aim of this paper is to report a case of a patient with HPS who was followed over a decade. A 2-and-a-half-year-old boy presented with recurrent respiratory tract infections and