Paraneoplastic Evans Syndrome in a Patient With Prostate Cancer With Small Cell Transformation.

Paraneoplastic syndromes are defined as tumor-associated indirect systemic effects. Prostate cancer-associated paraneoplastic syndromes typically have endocrine, neurologic, and dermatologic manifestations. Reports have suggested up to 70% of metastatic prostate cancers manifest as paraneoplastic entities. Although common in hematological malignancies, it is rare for prostate cancer and other solid tumors to be associated with immune-mediated cytopenias such as Evans syndrome. Based on our PubMed search for the keywords Evans syndrome and prostate cancer, only one other case has been reported in the literature with this association. We report the second such case in a 63-year-old male who initially presented with hemolytic anemia and thrombocytopenia. He was diagnosed with Evans syndrome with initial responses to both steroids and intravenous immunoglobulin. Extensive workup, including an eventual bone marrow biopsy, revealed metastatic prostate cancer with transformation to small cell neuroendocrine carcinoma.

First Case of Nodular Localized Primary Cutaneous Amyloidosis Treated With Bortezomib and Dexamethasone.

Nodular localized cutaneous amyloidosis is a rare form of cutaneous amyloidosis and is characterized by an extracellular deposition of insoluble amyloid fibrils which are either primarily cutaneous or a manifestation of an underlying systemic amyloidosis. Biopsy of the lesion is mandatory for the diagnosis, and histopathology shows diffuse amyloid deposits with plasmacytic infiltration. Apple-green birefringence characteristic of amyloidosis is observed when stained with Congo red and viewed under polarized light. Amyloid subtyping is done with laser microdissection followed by mass spectrometry. Majority of these lesions do not require any treatment but surgical excision, shave excision, laser therapy, and radiotherapy can be considered for symptomatic nodular localized primary cutaneous amyloidosis (NLPCA). We present a case of recurrent NLPCA in a 64-year-old woman who was treated with bortezomib and dexamethasone after failing several local therapies with excellent response.

Waldenstrom Macroglobulinemia Manifesting as Acute Kidney Injury and Bing-Neel Syndrome With Excellent Response to Ibrutinib.

Waldenstrom macroglobulinemia (WM) is a lymphoplasmacytic lymphoma associated with a monoclonal immunoglobulin M protein. Extranodal involvement in WM is not very common. In this article, we present a rare case of WM with kidney and central nervous system involvement. Bing-Neel syndrome is a distinct complication of WM where lymphoplasmacytic cells involve the central nervous system (CNS). Our patient was initially treated with dialysis and steroids with improvement in his kidney function. He was then started on systemic treatment with rituximab, cyclophosphamide, and dexamethasone with stable kidney function but persistent CNS symptoms. Due to rarity of cases, there is no standard treatment for Bing-Neel syndrome. His treatment was switched to ibrutinib with dramatic improvement in his CNS symptoms as well as radiological findings on magnetic resonance imaging.

Verrucous carcinoma arising in a long standing Buschke-Löwenstein tumor.

Giant condyloma acuminatum is a rare variant of genital warts also known as Buschke-Löwenstein tumor. It is characterized by a slow progression of exophytic, ulcerative, and cauliflower-shaped tumor with benign histological features. Verrucous carcinoma however is a rare variant of well-differentiated squamous cell carcinoma with limited metastatic potential.

Solitary Lytic Bone Metastasis: A Rare Presentation of Small Lymphocytic Leukemia.

Chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) is a hematologic malignancy characterized by an over accumulation of incompetent neoplastic lymphocytes. Bone metastasis in CLL/SLL is very rare. We report a case of a 76-year-old Caucasian female presented with an unresolving pulmonary infiltrate with mediastinal lymphadenopathy concerning for malignancy. Positron emission tomography (PET)/computed tomography (CT) showed an infiltrative mass in the mediastinum with diffuse uptake and a hypermetabolic mass within the left iliac bone. Transbronchial biopsy revealed morphology and features of SLL. However, with concern for another primary cancer, a CT-guided biopsy of the PET avid left iliac bone was performed and revealed bone and marrow with involvement of CLL/SLL similar to the chest lymphadenopathy. To our knowledge and after extensive review of medical literature, this is first reported case of SLL with solitary bone metastasis to the hip.

Chronic lymphocytic leukaemia with necrotic herpetic adenitis: an elusive clinical condition.

Herpes simplex virus (HSV) adenitis is a rare but important cause of morbidity in immunocompromised patients. Chronic lymphocytic leukaemia (CLL)/Small lymphocytic lymphoma (SLL) is an indolent disease which impairs the cellular and humoral immunity, predisposing patients to a myriad of infections. Clinically, herpetic adenitis can mimic large cell (Richter's) transformation in patients with CLL. To date, less than 30 cases of HSV adenitis have been reported in the literature. We report a case of a patient with CLL with no prior history of HSV infection, who presented with rapidly enlarging lymph nodes after initial response to idelalisib raising the suspicion of Richter's transformation. However, excisional biopsy of a lymph node revealed HSV adenitis along with CLL, which was confirmed by immunohistochemical staining.

Same difference: A pilot study of cyclin D1, bcl-2, AMACR, and ALDH-1 identifies significant differences in expression between primary colon adenocarcinoma and its metastases.

Tumor heterogeneity implies the possibility of significantly different expression of key pathways between primary and metastatic clones. Colon adenocarcinoma is one of the few tumors where current practice includes resection of primary and isolated organ metastases simultaneously without neoadjuvant therapy. We performed a pilot study on 28 cases of colon adenocarcinoma resected simultaneously with metastases in patients with no history of neoadjuvant therapy. We assayed matched primary and metastatic tumors from each patient with common diagnostic antibodies to Bcl-2, Cyclin D1, AMACR, and ALDH-1 by immunohistochemistry with semi-quantitative interpretation on archived formalin fixed, paraffin embedded samples. We were powered for large, consistent differences between primary and metastatic expression, and found 21 of 28 had a significant difference in expression of at least one of the four proteins, accounting for multiplicity of testing. Cyclin D1 had significantly more cases with differential metastatic:primary expression than would be expected by chance alone (p-value 0.0043), favoring higher expression in the metastatic sample. Bcl-2 and ALDH-1 had trends in this direction (p-value 0.078 each). Proportionately more cases with significant differences were identified when a liver metastasis was tested. We conclude differences in expression between metastatic and primary colon adenocarcinoma within the same patient exist, and may have therapeutic and biomarker testing consequences.

Leukemia cutis in a patient with chronic lymphocytic leukemia presenting as bilateral helical nodules.

Chronic lymphocytic leukemia, the most common adult leukemia worldwide, is considered an indolent but incurable non-Hodgkin lymphoma. Leukemia cutis is an uncommon manifestation of chronic lymphocytic leukemia. We present a case of an adult patient who presented with skin lesion of bilateral ears, which led to the diagnosis of chronic lymphocytic leukemia. We also reviewed the cases of auricular involvement in chronic lymphocytic leukemia patients reported in the literature. Local treatment is indicated in case of leukemia cutis; however, systemic treatment is recommended when there are systemic signs and symptoms. Better awareness of disease evolution and prompt diagnosis of this leukemia cutis of chronic lymphocytic leukemia will improve the effectiveness and outcome of its management.