Racial disparities in the incidence and survival outcomes in diffuse large B-cell lymphoma in adolescents and young adults.

Diffuse large B-cell Lymphoma (DLBCL) is an aggressive subtype of non-Hodgkin lymphoma (NHL). The disease generally occurs in older patients. Although at a lower prevalence, the disease also occurs in the adolescent and young adult group (AYA). There is paucity of data in the literature on racial and ethnic disparities in the incidence and survival outcomes of DLBCL in the AYA group. The objective of our study is to demonstrate the disparities in these outcomes. Utilizing SEER, we obtained data on patient demographics, incidence, and survival from 2000 to 2020. We observed statistically significant reduced incidence of DLBCL in all racial groups, except the non-Hispanic Asian and Pacific Islander group (NHAPI). The non-Hispanic Black group (NHB) had one of the lowest survival despite showing the largest decrease in incidence in DLBCL. The differences in the survival could be secondary to socioeconomic factors, however other reasons need to be explored. The increased incidence among the NHAPI group mirrors that of large population-based studies in East Asian countries, however, underlying reasons have not been elucidated.

Plasmablastic Lymphoma Presenting as Extensive Peritoneal and Retroperitoneal Nodules in an HIV-Positive Patient.

Plasmablastic lymphoma (PBL) is a rare but aggressive subtype of diffuse large B-cell lymphoma (DLBCL). The diagnosis of PBL is challenging as its features overlap with lymphoma and myeloma. The most common presentation involves the oral cavity/jaw in human immunodeficiency virus (HIV)-positive patients. It has also been reported in the gastrointestinal (GI) tract, lymph nodes, and soft tissues. Usually, if PBL involves the GI tract, it presents as a gut tumor mass. In this report, we present an HIV-positive patient with PBL presenting with multiple peritoneal nodules. To our knowledge, this is the first case of PBL presenting as multiple peritoneal and retroperitoneal nodules in an HIV-positive patient. This case emphasizes the rare presentation of a rare malignancy, difficulties in establishing a diagnosis, and the importance of proper and timely management.

Aggressive Diffuse Intermediate Size B-Cell Lymphoma With P53 Mutation Presented as Primary Bone Marrow Lymphoma.

Primary bone marrow lymphoma (PBML) is a disease entity in which lymphoma primarily originates in the bone marrow without signs of involvement of lymph nodes, spleen, liver, or any other organs, and excludes leukemia/lymphoma. PBML has been a rare presentation of malignant lymphoma, and most of the cases have a poor prognosis and require rapid diagnoses and treatments. Among all PBMLs, diffuse large B-cell lymphoma (DLBCL) is the most common pathological subtype. Over 25 years and from 7 institutions, the International Extranodal Lymphoma Study Group retrospectively collected PBML cases and, in 2012, published these 21 cases, including 19 cases of B-cell lymphoma and 2 cases of peripheral T-cell lymphoma. Among the B-cell types, DLBCL accounted for 79% and follicular lymphoma (FL) for 21%. DLBCLs were characterized by the existence of large cells. In this article, we present a rare case of high-grade aggressive type with P53 mutation, intermediate-sized B-cell lymphoma, excluded FL by the absence of FL lymphoma markers, presented as PBML. Our patient had rapid progression and succumbed to the disease shortly after diagnosis. Upon literature review, 62 B-cell lymphoma cases were identified that presented as PBML (51 high-grade and 11 low-grade)-mostly case reports. Among these, only one case was reported as intermediate-sized DLBCL-like lymphoma but not with aggressive features. Our case represents the first case of aggressive intermediate-sized lymphoma, not a FL, with P53 mutation, highly elevated lactate dehydrogenase, and Ki-67 presented as PBML. Such a profile would need to be quickly recognized and aggressive treatment applied, such as CART (chimeric antigen receptor T-cells) therapy or DA-EPOCH-R (dose-adjusted EPOCH [etoposide-prednisone-oncovin-cyclophosphamide-hydroxydaunorubicin] and rituximab) with or without venetoclax.

Unexplained chronic cytopenia: is it idiopathic cytopenia of undetermined significance or myelodysplastic syndrome.

Idiopathic cytopenia is a condition where there is a decrease in peripheral blood counts causing either anaemia, leucopoenia and thrombocytopaenia. Most cases of cytopenia reveal a cause on further workup. But very rarely, in some cases, a definitive cause could not be identified. Unexplained cytopenia becomes challenging and poses difficulty in diagnosis and management. Discriminating these groups of bone marrow failure disorders from myelodysplastic syndrome (MDS) becomes an important clinical question. We describe a case of a middle-aged Hispanic woman who presented with pancytopenia and on extensive workup did not reveal any specific cause. Her bone marrow examination revealed severely reduced megakaryocytes but with normal haemopoiesis of other lineages. Cytogenetics, flow cytometry, comprehensive next-generation whole genomic analysis did not reveal any abnormalities. She fit the criteria for idiopathic cytopenia of undetermined significance rather than MDS. She remained asymptomatic and her counts never improved with immunosuppressives or thrombopoietin mimetics.

Chronic myelomonocytic leukemia as a transformation from polycythemia vera.

The hallmark of Polycythemia vera (PV) is the presence of JAK2V617F mutation and increased RBC mass. Chronic myelomonocytic leukemia (CMML) is defined as persistent blood absolute monocyte count (AMC) >/= 1 × 109/L for at least 3 months with myeloid cell dysplasia. Few cases of evolved CMML from PV have been described. We present a case of PV that progressed to CMML. We demonstrated the CMML clone was most likely derived from PV- JAK2V617F clone. This clone carried a complex genetic mutations of ASXL1, RUNX1, SRSF2 and TET2, NRAS, KRAS, plus CMML cells were of the classical phenotype CD14+ CD16-by flow cytometry.

Uncommon Presentation of Metastatic Squamous Cell Carcinoma of the Skin and Treatment Challenges.

BACKGROUND Squamous cell carcinoma is one of the most common keratinocytic skin cancers, the other being basal cell carcinoma. It is the second most common skin cancer after melanoma. Cutaneous squamous cell carcinoma is mostly a localized disease. The metastatic presentation is rare even in the presence of invasive disease. The metastatic potential depends on the presence of high-risk features at the time of diagnosis. Lung, liver, and bone are the frequent sites of metastasis. Local and locoregional disease undergoes excision with or without adjuvant radiation. However, we lack proper treatment paradigms for this metastatic disease. CASE REPORT We are reporting a case of an elderly female with a history of high-risk localized cutaneous squamous cell carcinoma treated with complete local excision and radiation presenting 5 years later with extensive disease to the lung and liver, abdominal nodes, and spinal fracture. The patient was not a candidate for chemotherapy due to kidney failure. On the basis of ongoing separate trials on different immunotherapies, she was started on nivolumab. CONCLUSIONS Treating metastatic cutaneous squamous cell carcinoma is a challenge considering the absence of phase III trials due to the rarity of this disease. Historically, platinum with or without 5-FU (fluorouracil), bleomycin, doxorubicin, and retinoic acid were used with variable responses. Data on epidermal growth factor receptor (EGFR) inhibitors on EGFR expressing tumors are available. However, even with the most recent reports on immunotherapy in patients with high programmed death-1 expression or high mutation burden, it is difficult to achieve good response.

Superwarfarin Exposure: An Important Uncommon Cause of Painless Bleeding.

Painless bleeding in a patient presenting from the community with elevated coagulation studies rarely makes the physicians suspect superwarfarin or rodenticide poisoning. Although a significant number of superwarfarin exposure cases are diagnosed every year, we believe there appears to be delay in diagnosis and confusion in determining what is the ideal way to treat and monitor these patients during the management. This is the first thorough literature review of all the reported cases of superwarfarin poisoning which also studied the clinical presentation, management and follow-up patterns. We present a 70-year-old man who presented to the emergency room with epistaxis, melena, cola-colored urine with elevated prothrombin time (PT), activated partial thromboplastin time (aPTT) and international normalized ratio (INR). Mixing studies showed complete correction of coagulopathy indicative of factor deficiency. Additional history revealed that the patient had arguments with family member at home and made us suspect superwarfarin exposure. Qualitative brodifacoum testing was positive and was managed with fresh frozen plasma and high doses of vitamin K1 (phytomenadione) with serial monitoring of INR and clinical symptoms. Superwarfarin poisoning should be considered in the differential diagnosis of a patient who presents with above clinical and laboratory profile especially in the absence of any history of coagulopathy or anticoagulant use. We want to raise public and especially physician awareness that history taking, early diagnosis and managing in right clinical setting play a significant role in survival of these patients.

Primary Pulmonary Diffuse Large B Cell Non-Hodgkin's Lymphoma: A Case Report and Literature Review.

BACKGROUND Primary pulmonary diffuse large B cell lymphoma (DLBCL) is extremely rare neoplasm representing only 0.5-1% of primary pulmonary malignancies. These patients usually have non-specific clinical presentation and radiological findings. Therefore, it is important to increase awareness of this rare disease, as the correct characterization of the tumors will have therapeutic and prognostic implications. CASE REPORT We present the case of a middle-aged Hispanic woman with chronic cough and an abnormal chest X-ray revealing a lung mass, who was found to have primary pulmonary DLBCL. She underwent 6 cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) therapy and attained complete remission. CONCLUSIONS With its non-specific presentation, the diagnosis of primary pulmonary DLBCL is very challenging and often leads to misdiagnosis or delayed diagnosis. The pathogenesis of primary pulmonary DLBCL is still poorly understood. The choice of treatment approach should be based on the biological characteristic of the tumor, stage, and performance status.

Synchronous Small Cell Neuroendocrine Carcinoma and Adenocarcinoma of the Colon: A Link for Common Stem Cell Origin?

Synchronous carcinomas have been recognized for over a century, with synchronous primary adenocarcinoma of the colon reported to range from 2-11% of cases involving this type of malignancy. Small cell carcinomas occur frequently with colorectal adenomas; however, despite these reports and a known adenoma-to-carcinoma sequence, scarce literature exists on synchronous colorectal adenocarcinoma and small cell carcinomas. We present a rare cancer of synchronous small cell neuroendocrine carcinomas and discuss a possible link between these two cancers.

Hairy cell leukemia in a patient with situs inversus totalis: an extremely rare combination.

Hairy cell leukemia is a rare cancer of the blood. The occurrence of hairy cell leukemia with another very rare genetic disorder makes us question whether it is just a coincidence. This article reports the first case of hairy cell leukemia in a patient with situs inversus totalis in western literature. There have been studies into the pathogenesis of situs inversus totalis that suggest it is caused by the failure of embryonic cells to properly rotate during embryogenesis. On the molecular level, the nodal cilia, which are responsible for embryonic rotation, are built by transport through the KIF3 complex - a kinesin superfamily of molecular motors. The KIF3 complex is also responsible for N-cadherin movement in cells. Furthermore, it is well known that these cell adhesion molecules play an important role in carcinogenesis and its progression. This report attempts to link the rare conditions and propose a possible genetic relationship between the two.

Obesity, metabolic factors, and colorectal adenomas: a retrospective study in a racially diverse New York State Hospital.

OBJECTIVE: We studied a racially diverse population and the relationship with colorectal adenomas (CA) further looking for risks related to BMI and metabolic factors. DESIGNS: Seven hundred seventy-nine patients who underwent screening colonoscopies between 2007 and 2009 meeting exclusion criteria were included. To evaluate the association between race, BMI, and other metabolic factors with having one or more CA detected at colonoscopy, adjusted odds ratios and 95 % CI were estimated using unconditional logistic regression models. OUTCOMES: CA were detected in 167 out of 779 (21.4 %) patients. Compared to Whites, Hispanics were less likely to have one or more adenomas detected during a screening colonoscopy (OR = 0.52, 95 % CI, 0.31-0.88; p = 0.01). There was no significant statistical difference between Blacks and Whites, or other races and Whites. There was an association between the presence of CA and smoking (OR = 1.57, 95 % CI, 1.02-2.43; p = 0.04). CONCLUSION: Our results showed that Hispanics were less likely to have an adenoma detected during a screening colonoscopy than Whites. No statistical significant difference was found between patients with metabolic factors and the presence of colorectal adenoma.

Budd-Chiari syndrome induced by stage IV rectal carcinoid.

Budd-Chiari syndrome (BCS) is defined as an obstruction of the hepatic venous outflow anywhere from the small hepatic veins to the suprahepatic inferior vena cava. In this study, a rare case of BCS induced by a metastatic rectal carcinoid is presented. A 57-year-old African American woman with stage IV rectal carcinoid presented with right upper quadrant pain, associated with decreased appetite and weight loss >13 kg over 2 months. Computed tomography scan with contrast enhancement revealed filling defects in the left and middle hepatic veins extending into the suprahepatic inferior vena cava to the junction of the right atrium, suggesting BCS. Thrombophilia workup was negative, and no signs of liver cirrhosis or portal hypertension were found. A hepatitis profile workup yielded negative results. This is the first reported case of BCS that is associated with a metastatic rectal carcinoid. More research is needed to identify the mechanism leading to thrombogenesis in carcinoid tumors.

Germ cell cancer presenting as gastrointestinal bleeding and developing brain metastases: case report and review of the literature.

This paper describes a rare case of germ cell cancer with duodenum, brain and lung metastases. The patient presented with melena and left testicle enlargement. Orchiectomy revealed mixed germ cell cancer, enteroscopy revealed duodenal choriocarcinoma, and chest x-ray and computed tomography (CT) showed bilateral lung metastases. The patient received and tolerated cisplatinum-based chemotherapy, and responded well. However, he developed seizures 3 months later. MRI showed brain metastases and he was treated with whole-brain radiation. One month later, he developed progressive dyspnea. Chest CT showed worsening lung metastases. He received second-line chemotherapy, but died due to multiorgan failure. Germ cell cancer with nonpulmonary metastases has poor prognosis and the management of these patients requires a multimodal approach. Head CT should be considered as routine screening for all germ cell cancer patients on initial diagnosis and brain MRI should be considered for high-risk patients (with an embryo- or choriocarcinoma histology, dramatically elevated ß-human chorionic gonadotropin and lung involvement).

Inflammatory malignant fibrous histiocytoma associated with leukemoid reaction or leukocytosis: a comprehensive review.

Inflammatory malignant fibrous histiocytoma (IMFH) associated with leukemoid reaction (LR)/leukocytosis is a rare entity. In this paper, we search PubMed for all known cases of IMFH associated with LR/leukocytosis in an attempt to draw conclusions about this variant's response to treatments and its pathophysiology. Medline electronic database was searched using key words such as malignant fibrous histiocytoma, leukemoid reaction, and leukocytosis. A total of 16 patients were found, twelve males (75%) and 4 female (25%), with a mean age of 62.6 years, ranging from 47 to 77. The mean survival was 770 days, ranging from 14 to 6570 days. Four patients were alive at last follow-up: 6570 days, 1095 days, 335 days, and 180 days, respectively. Of the 12 patients that expired, death occurred approximately 92 days after the onset of LR or leukocytosis, ranging from 3 to 334 days. We conclude that IMFH associated with LR/leukocytosis does not completely respond to chemoradiation. Overproduction of growth factors and cytokines by IMFH cells and their interactions with the inflammatory infiltrate seem to promote immunological effector cell's dysfunction and substantiate the development and growth of this neoplasm. A clear understanding of these molecular pathways is crucial in order to identify targets for potential therapy.

Cutaneous inflammatory malignant fibrous histiocytoma presenting with a leukemoid reaction: a case report and review of the literature.

Malignant fibrous histiocytoma (MFH) is the most common sarcoma found in adults. We discuss a case of inflammatory MFH of dermal/epidermal origin presenting with a severe leukemoid reaction (LR). A 60 years old white male presented to hematology/oncology clinic complaining of mild shortness of breath on exertion. Past medical history was remarkable for removal of a left upper extremity necrotic mass 4.4 × 3 × 3 cm. Microscopy of the specimen showed clear surgical margin, and tumor cells restricted to the dermis without lymphovascular invasion. Immunohistochemestry was positive for CD 68 and CD 99. Chest x-ray was negative for metastatic disease. White blood cell count was 109.4 k/mm(3) with 24 k/mm(3) band neutrophils, and absolute neutrophil count of 69 k/mm(3). CT scan of the thorax revealed numerous bilateral pulmonary nodules suspicious for metastasis. Based on these findings patient was diagnosed with metastatic cutaneous IMFH associated with a LR. Following review of medical literature, this appears to be the first reported case of inflammatory cutaneous MFH associated with LR. This histological variant is rare, and carries a poor prognosis. Thus, we would like to emphasize the need for investigating alternative therapies capable of improving the survival of these patients.

Hepatocellular carcinoma presenting as an incidental isolated malignant portal vein thrombosis.

INTRODUCTION: Portal vein thrombosis is frequently associated with hepatocellular carcinoma (HCC). Tumor invasion into the portal vein by direct venous extension or metastasis occurs in up to 70% of HCC patients (Cedrone et al., Liver 16:94-8, 1996). However, presentation as an isolated malignant portal vein thrombosis without any evidence of obvious hepatoma-like lesions in the liver by imaging studies is extremely uncommon. We present an unusual case of HCC presenting as a malignant portal vein thrombus, proven on biopsy of the thrombus without any evidence of primary liver lesion. This, to our knowledge, is the first case of HCC presenting as an incidental isolated malignant portal vein thrombosis. The importance of doing delayed enhancement imaging studies to rule out malignant portal vein thrombosis is emphasized. CASE REPORT: A 60-year-old man presented with acute substernal chest pain. Physical examination revealed icterus. Examination of the abdomen did not reveal any organomegaly. Liver function test revealed a predominantly conjugated bilirubinemia. Abdominal sonogram revealed thrombosis and occlusion of the posterior right portal vein. Liver parenchyma was homogenous with no intrahepatic mass. Computed tomography (CT) of the abdomen and pelvis after administration of oral and intravenous contrast with delayed views revealed arterial enhancement of the right portal vein thrombus with delayed washout. MRI of the abdomen with gadolinium confirmed the right portal vein thrombus without focal hepatic mass. Aspiration of the right portal vein thrombus under CT guidance revealed hepatocellular carcinoma which was confirmed by immunohistochemistry. Serum alpha-fetoprotein level was very high. Patient was started on sorafenib with subsequent decrease in alpha-fetoprotein level. He was doing well till the date of this report. DISCUSSION: This unusual case of hepatocellular carcinoma presenting as an incidental malignant portal vein thrombosis without any primary liver lesion is extremely rare. Other reported cases of malignant portal vein thrombosis have been in patients with underlying hepatoma, cirrhosis, or with intrabiliary hepatocelluar carcinoma. In the clinical setting of portal vein thrombosis, imaging studies showing enhancement of the thrombus in the arterial phase are important in leading to the diagnosis of malignancy.

Synchronous Duodenal Carcinoid and Adenocarcinoma of the Colon.

Carcinoid tumors are a histological subtype of well differentiated, low to intermediate grade, slow-growing neuroendocrine malignancies capable of secreting bioactive peptides, such as 5-hydroxytryptamine (5-HT, serotonin), chromogranin-A and chromogranin-C. Here we present a case of a duodenal carcinoid that simultaneously occurred with adenocarcinoma of the colon. A 59-year-old male with a past medical history of hepatitis C and hypertension presented complaining of worsening abdominal pain associated with 2 - 3 episodes per week of bright red blood per rectum for the past month. He also reported a 20 pounds weight loss in the last 6 months. Social history was significant for a 15 pack year history. Vitals on admission were within normal limits. Physical exam was significant for right upper quadrant tenderness without guarding, rebound, or organomegaly. Rectal exam revealed no blood or masses. Laboratory results showed iron deficiency anemia with hemoglobin of 9.6 K/mm3. Esophagogastroduodenoscopy revealed a 4 mm duodenal polyp. Colonoscopy was terminated early secondary to a large circumferential obstructing mass found in the descending colon. Immunohistochemistry of the duodenal biopsy was positive for synaptophysin and chromogranin-A; consistent with the diagnosis of stage I carcinoid tumor. Biopsy results of the colonic mass showed a stage I well-differentiated adenocarcinoma. The patient underwent a left colectomy and partial duodenectomy; he remains in remission after 2 year of close follow up. When the diagnosis of small bowel carcinoid is made, further screening for other primary neoplasms should be sought to prevent potential late stage diagnosis of synchronous malignancies. This is crucial because patients' demise usually result from the associate tumor and not the carcinoid component. Finally, we would like to raise clinician's awareness regarding the incidence of this entity since some of the studies suggest that it is more common than it was previously thought.