Yehuda Lebowicz

Double-Hit Large B Cell Lymphoma

Diffuse large B cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL), accounting for approximately 25% of NHL cases. It is a heterogeneous group of diseases. BCL2, BCL6, and MYC are the most frequent mutated genes in DLBCL. Double-hit lymphoma (DHL) is an aggressive form of DLBCL with an unmet treatment need, in which MYC rearrangement is present with either BCL2 or BCL6 rearrangement. Patients typically present with a rapidly growing mass with B symptoms. DHL has been linked to very poor outcomes when treated with RCHOP chemotherapy. Dual-expressor lymphoma is a form of DLBCL with overexpression of MYC and BCL2/BCL6. There is a paucity of prospective trials evaluating the treatment of DHL. Retrospective series suggest that more aggressive treatment regimens such as DA-EPOCH and hyper CVAD may be more efficacious. However, there remains a lack of consensus regarding optimal treatment for DHL. Further clinical trials, including novel agents, are needed for improvement in outcomes.

Relapsed Acinic Cell Carcinoma of the Parotid Gland With Diffuse Distant Metastasis Case Report With Literature Review

Acinic cell carcinoma (ACC) is an uncommon salivary gland neoplasm that generally displays an indolent growth pattern. Most cases arise in the major glands, particularly the parotid. However, it can arise from minor salivary glands in the oral cavity and aero-digestive tract. Although ACC is generally a low-grade malignant tumor, poorly differentiated and high-grade transformed variants exhibit a propensity for late recurrence and metastasis. There are no adequate clinical trials that define the optimal approach to patients with metastatic salivary gland tumors due to its rarity. Systemic therapy is reserved for cases where local therapy, such as radiation or metastasectomy, is not appropriate. Nevertheless, there is insufficient data in the literature regarding the chemotherapy of choice for metastatic ACC. In this article, we report a case of metastatic ACC of the right parotid gland that progressed on carboplatin and paclitaxel after partial response followed by doxorubicin and is currently on checkpoint inhibitor treatment.

An Unusual Presentation of Isolated Leptomeningeal Disease in Carcinoma of Unknown Primary With Pancreatic Features

Leptomeningeal disease (LMD) can occur in a small percentage of patients with active metastatic cancer. However, we report a case of LMD occurring during disease remission in a patient with carcinoma of unknown primary with panreaticobiliary features. A 45-year-old woman was found with mediastinal and abdominal lymphadenopathy with lymph node biopsy consistent with adenocarcinoma, expressing immunomarkers CK7, CK20, and Ca19-9 along with markedly elevated serum Ca19-9 level. The patient was started on a pancreatic cancer directed chemotherapy regimen of Folfirinox (5-fluorouracil, leucovorin, oxaliplatin, irinotecan) and achieved complete response. She was then noted to have slowly rising Ca19-9 level that did not correlate with her lack of evidence of systemic disease progression. Eventually, she presented with neurologic symptoms and was found on imaging to have isolated LMD.

A Rare Case of Metastases from a High-grade Astrocytoma to the Pleura, Bones, and Liver within Six Months of Diagnosis

High grade astrocytomas such as anaplastic astrocytoma and glioblastoma multiforme are aggressive central nervous system malignancies with a poor prognosis. Due to shortened survival times, their devastating effects are usually localized intracranially and rarely metastasize outside of the central nervous system. When metastases occur, they usually present in patients with longer survival times and they typically coincide with a primary site recurrence. We present a rare case of metastases from a high-grade astrocytoma/glioblastoma to the pleura, bones and liver within six months of diagnosis, without primary site recurrence.

Extraskeletal Chondrosarcoma: Long-term Follow-up of a Patient with Metastatic Disease

Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma with an indolent course and poor response to systemic treatment. We present a case of a 53-year-old male who presented with right gluteal extraskeletal myxoid chondrosarcoma. He was treated with wide local excision after receiving 50 Gray of neoadjuvant radiation therapy. Three years later he was found to have a left lower lobe lung nodule that was slowly increasing in size. He underwent a left lower lobectomy and the nodule was confirmed to be consistent with the patient’s history of EMC. One year later, lung imaging showed multiple small nodules bilaterally consistent with metastatic disease. The patient opted for watchful waiting approach. Routine follow-up imaging for four years shows a very slow progression of his disease burden. He continues to be asymptomatic. This case demonstrates the natural course of EMC and argues in favor of the watchful waiting approach in treating this disease.

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.

Primary Retroperitoneal Melanoma Presented in a Rare Extracutaneous Site for Malignant Melanoma

Malignant melanoma, as the name implies, is a malignant tumor of melanocytes, found in the skin, eyes, meningeal lining and the mucosal epithelium of the aero-digestive and genitourinary tracts. Malignant melanoma is typically skin malignancy, which rarely presents at extracutaneous site. Here we present a rare case of primary retroperitoneal melanoma and review the findings in comparison with other cases described in literature.

Splenic infarction: An uncommon presentation of acute infectious mononucleosis

Splenic infarction is a relatively uncommon diagnosis. It occurs when the splenic artery or one of its sub-branches is occluded with an infected or bland embolus or clot. Splenic infarction may be caused by atrial fibrillation, bacterial endocarditis, sickle cell disease, antiphospholipid syndrome, and trauma whereas an infectious etiology is uncommon. It is considered a rare presentation of acute infectious mononucleosis. Currently, its pathogenesis is still unclear. We describe a 24-year-old African American female who was admitted for evaluation of left-sided chest pain. Chest imaging, an abdominal ultrasound, and initial laboratory data were normal, followed by a negative hypercoagulability panel. Signs and symptoms of infectious etiology were absent, however, both IgM and IgG antibodies for Ebstein-Barr virus (EBV) viral capsid antigen were high. Contrast-enhanced abdominal computed tomography revealed splenomegaly and multiple infarcts in the spleen, which eventually led to the diagnosis of infectious mononucleosis-associated splenic infarction, resolving the diagnostic dilemma.

Cutaneous presentation of Double Hit Lymphoma A Case Report With Review of the Literature

Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL), representing approximately 25% of diagnosed NHL. DLBCL is heterogeneous disease both clinically and genetically. The 3 most common chromosomal translocations in DLBCL involve the oncogenes BCL2, BCL6, and MYC. Double hit (DH) DLBCL is an aggressive form in which MYC rearrangement is associated with either BCL2 or BCL6 rearrangement. Patients typically present with a rapidly growing mass, often with B symptoms. Extranodal disease is often present. Though there is a paucity of prospective trials in this subtype, double hit lymphoma (DHL) has been linked to very poor outcomes when patients are treated with standard R-CHOP. There is, therefore, a lack of consensus regarding the standard treatment for DHL. Several retrospective analyses have been conducted to help guide treatment of this disease. These suggest that DA EPOCH-R may be the most promising regimen and that achievement of complete resolution predicts better long-term outcomes.

Metastatic Sarcomatoid Carcinoma of the Small Intestine: a Case Report of Rare Tumor with Literature Review

Sarcomatoid carcinoma (SCA) is an uncommon variant of small intestinal carcinoma with both carcinomatous and sarcomatous features [1–3]. Gastrointestinal SCA has been described in the gall bladder, stomach, esophagus, and colon, but is rarely reported in the small bowel, with less than 30 cases reported to date in the literature in English [1]. The natural history of this unusual tumor and the best treatment approach are unknown. A patient with jejunal SCA usually presents with a large tumor which is likely a reason for the poor outcome as compared to esophageal and gastric ones which usually present early with small tumor causing obstructive symptoms. We present a case of SCA arising from the jejunal mucosa with findings from both morphological and immunohistochemical studies.