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Competing Interests: Conflicts of interest None.

Metastatic clear cell carcinoma (mCCC) is a rare histological subtype of cancer with ovarian and renal origins most common primary sites. Cancer of unknown primary origin (CUP) is a rare type of cancer in the United States and the most common histologic subtypes are adenocarcinoma, squamous cell cancer, and neuroendocrine cancer. We are presenting a rare case of an 86-year-old female patient with mCCC of unknown origin, biopsy and staining showed renal and ovarian in the differential of primary cancer type. However, the patient did not survive the aggressive nature of mCCC and was unable to get any trials of chemotherapy. Primary sites of adenocarcinoma of unknown origin are most common in the breast, lung, pancreas, prostate, colon, and liver. In most cases, empiric chemotherapy with platinum-based agents is the standard of care but needs more data to manage CUP, making it difficult to identify the primary site., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Al Omour et al.)

Colorectal cancer (CRC) is the second-leading cause of cancer-related deaths globally. BRAF mutation is present in about 10% of CRC patients and is associated with a poor response to chemotherapy. These patients have a relatively poor prognosis. This review aims to assess the efficacy and safety of BRAF inhibitors in BRAF - mutated CRC patients. A literature search was performed on PubMed and Embase, and clinical trials relevant to BRAF inhibitors in CRC were included. Data were extracted for efficacy and safety variables. Two randomized clinical trials ( n = 765) and eight non-randomized trials ( n = 281) were included based on inclusion criteria. In RCTs, an overall response was reported in 23% of the patients treated with BRAF inhibitor-based regimens compared to 2.5% with control regimens. The hazard ratio of overall survival was also significantly better with triplet encorafenib therapy at 0.52 (95% CI = 0.39-0.70). In single-arm trials, ORR was 17% and 34% in two-drug and three-drug regimens, respectively. BRAF inhibitor-based regimens were safe and effective in the treatment of BRAF -mutated CRC. Large-scale randomized trials are needed to find a suitable population for each regimen. PROSPERO registration No. CRD42023471627.

In this report, we present the case of a patient with an uncommon triple diagnosis of (1) follicular (nodular) lymphoma, (2) nodular lymphocyte predominant Hodgkin lymphoma, and (3) diffuse large B-cell lymphoma non-germinal center B-cell (non-GCB) subtype. Although transformation of follicular lymphoma and nodular lymphocyte predominant lymphoma to more aggressive forms such as diffuse large B-cell lymphoma is possible; it generally happens many years after diagnosis. Moreover, there have been reported cases of follicular lymphoma with transformation and nodular lymphocyte predominant Hodgkin lymphoma with transformation at the time of diagnosis, but it is very uncommon to see all three present on initial diagnosis. Our patient presented with a large right axillary mass, which, upon excisional biopsy and subsequent histology, showed the aforementioned lymphomas. The patient did not present with a prodrome of any symptoms except intermittent night sweats. The unique aspect of our case is that transformation and all three lymphomas were seen at the time of diagnosis. The R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy regimen is still the standard method of treatment as it has been shown to be effective in treating follicular lymphoma and nodular lymphocyte predominant Hodgkin lymphoma with and without transformation. However, there is insufficient literature on its efficacy when all three are present concurrently., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Rahim et al.)

Hairy cell leukemia (HCL) is a rare neoplasm of the B-cell lineage that is characterized by an indolent course and infiltration of the spleen, the bone marrow, and the reticuloendothelial system. Splenectomy is considered an effective treatment for peripheral cytopenia in patients with HCL. Hepatic involvement of hairy cells with infiltration of the sinusoidal endothelial cells is rarely reported in the literature and is not well understood. We present the case of an 88-year-old male with a history of traumatic splenectomy who was found to have a relapse of classic hairy cell leukemia within the hepatic portal system., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Dhanesar et al.)

Background: Acute myeloid leukemia (AML) is a hematological malignancy due to anomalous differentiation and proliferation of hematopoietic stem cells with myeloid blast buildup. Induction chemotherapy is considered the first line of treatment in most patients with AML. However, targeted therapy in the form of FLT-3, IDH, BCL-2, and immune checkpoint inhibitors, can be considered as the first line depending on their molecular profile, resistance to chemotherapy, comorbidities, etc. This review aims to assess the tolerability and efficacy of isocitrate dehydrogenase (IDH) inhibitors in AML., Methods: We searched Medline, WOS, Embase, and clinicaltrials.gov. PRISMA guidelines were followed in this systematic review. 3327 articles were screened, and 9 clinical trials (N = 1119) were included., Results: In randomized clinical trials (RCTs), objective response (OR) was reported in 63-74% of the patients with IDH inhibitors + azacitidine as compared to 19-36 % of the patients with azacitidine monotherapy in newly diagnosed (ND) medically unfit patients. Survival rates were significantly improved with the use of ivosidenib. OR was reported in 39.1-46 % of the patients who relapsed/refractory to chemotherapy. ≥Grade 3 IDH differentiation syndrome and QT prolongation were reported in 3.9-10 % and 2-10 % of the patients, respectively., Conclusion: IDH inhibitors (ivosidenib for IDH-1 and enasidenib for IDH-2) are safe and effective in treating ND medically unfit or relapsed refractory patients with IDH mutation. However, no survival benefit was reported with enasidenib. More randomized multicenter double-blinded clinical studies are needed to confirm these results and compare them with other targeting agents., Competing Interests: Declaration of Competing Interest Faiz Anwer reports institutional research funding from Allogene Therapeutics, Celgene,GlaxoSmithKline, and Bristol-Myers Squibb; honoraria from Bristol-Myers Squibb and JanssenPharmaceuticals; and participation on a data safety monitoring board or advisory board at Bristol-MyersSquibb and Janssen Pharmaceuticals outside the submitted work. Aurore Perrot reports honoraria fromAbbVie, Amgen, Bristol-Myers Squibb/Celgene, GlaxoSmithKline, Janssen Pharmaceuticals, Sanofi, andTakeda; and participation on a data safety monitoring board or advisory board at Janssen Pharmaceuticalsoutside the submitted work. The remaining authors have no disclosures., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Primitive neuroectodermal tumors (PNET) represent malignant neuroectodermal tumors composed of small round cells. They can be differentiated between originating from the peripheral nervous system or the central nervous system. Peripheral PNET (pPNET) can be further subclassified as one of the Ewing family tumors (EFT). Although rare, EFT can originate in the female genital tract and pelvic region. Here, we present a case of a middle-aged female with PNET masses in her uterus, abdomen, and hepatic lobes. We discuss the diagnostic modalities, including immunohistochemistry, histopathology, and imaging findings associated with this rare malignancy., (© 2023 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Immune thrombocytopenic purpura (ITP) is an acquired antibody or cell-mediated platelet damage or decreased platelet production. Steroids, IV immunoglobulins (IVIG), and Rho-anti-D antibodies are the commonly used initial treatments for ITP. However, many ITP patients either do not respond or do not maintain a response to initial therapy. Splenectomy, rituximab, and thrombomimetics are the commonly used second-line treatment. More treatment options include tyrosine kinases inhibitors (TKI), including spleen tyrosine kinase (Syk) and Bruton's tyrosine kinase (BTK) inhibitors. This review aims to assess the safety and efficacy of TKIs. Methods: Literature was searched on PubMed, Embase, WOS, and clinicaltrials.gov using keywords, "tyrosine kinase" and "idiopathic thrombocytopenic purpura". PRISMA guidelines were followed. Results: In total, 4 clinical trials were included with 255 adult patients with relapsed/refractory ITP. In all, 101 (39.6%) patients were treated with fostamatinib, 60 (23%) patients with rilzabrutinib, and 34 (13%) with HMPL-523. Patients treated with fostamatinib achieved a stable response (SR) and overall response (OR) in 18/101 (17.8%) and 43/101 (42.5%) of the patients, respectively, while SR and OR were achieved in 1/49 (2%) and 7/49 (14%) of the patients, respectively, in the placebo group. Patients treated with HMPL-523 (300 mg dose expansion) achieved an SR and OR in 5/20 (25%) and 11/20 (55%) of the patients, respectively, while SR and OR were achieved in 1/11 (9%) of the patients treated with the placebo. Patients treated with rilzabrutinib achieved an SR in 17/60 (28%) patients. Dizziness (1%), hypertension (2%), diarrhea (1%), and neutropenia (1%) were serious adverse events in fostamatinib patients. Rilzabrutinib or HMPL-523 patients did not require a dose reduction due to drug-related adverse effects. Conclusions: Rilzabrutinib, fostamatinib, and HMPL-523 were safe and effective in the treatment of relapsed/refractory ITP.

BACKGROUND Cutaneous metastatic breast cancer is a rare manifestation. Causes include vascular or lymphatic spread or iatrogenic mechanisms following surgery. A sub-type of this disease process, "Carcinoma Erysipeloides," represents subcutaneous and dermal tissue layer invasion via lymphatic spread. Diagnosis can be challenging, and therefore, obtaining a thorough history and physical, with careful inspection of prior surgical scars is essential for an accurate diagnosis. Lesions present in variable ways, including papules, plaques, ulcerations, nodules, crusting, or fungating masses, with common locations in the chest, scalp, abdomen, and less commonly the arms. When carcinoma erysipeloides is identified, it is imperative to evaluate for distant metastases. Recent literature has identified benefits with trastuzumab deruxtecan therapy instead of trastuzumab emtansine, with decreased progression rates and decreased mortality rates. Metastasis to the skin can indicate advanced disease; however, this metastatic site may be preferable to visceral organs or bones in terms of prognosis. CASE REPORT We present a rare manifestation of metastatic breast cancer in 45-year-old Hispanic woman, status post neoadjuvant chemotherapy and radical cystectomy on maintenance trastuzumab and pertuzumab. We discuss the clinical presentation variability, keys to diagnosis, treatment considerations, and outcomes for this unique patient population. CONCLUSIONS Carcinoma erysipeloides varies in clinical presentation, especially when patients develop exclusive skin lesions. We identify common etiologies for this progression of disease and discuss combination therapy which has demonstrated a reduction in mortality in this patient population.

Gastric adenocarcinoma of the cervix (GAC) represents a rare mucinous endocervical cancer unrelated to human papillomavirus (HPV). GAC has been found to comprise approximately 10% of cervical adenocarcinomas internationally. As more cases have been identified, GAC has been further classified into subtypes such as poorly differentiated versus well-differentiated (also referred to as mucinous or adenoma malignum). This cancer coined the term "gastric" subtype due to its similarity to the pancreaticobiliary and gastric tissue lining. With limited data and similar histological and genetic features of GAC, this malignancy poses a challenge for clinicians when differentiating between metastasis from the gastrointestinal tract and GAC. Here, we present a case of a 55-year-old female who presented with postmenopausal bleeding and was found to have stage IA1 endocervical adenocarcinoma of gastric subtype. The purpose of this article is to introduce a rare type of gastric adenocarcinoma with a unique site of origin in order to better understand this disease process and potentially help clinicians better diagnose and treat patients with this malignancy in the future., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Mekheal et al.)

BACKGROUND Male breast cancer represents a rare malignancy with identifiable risk factors, including genetics, radiation exposure, liver dysfunction, and concomitant diagnosis of Klinefelter syndrome. Gynecomastia can commonly present in these patients, and despite increased estrogen levels in adipose breast tissue, gynecomastia has not been proven to be a significant risk factor for carcinoma development. Male patients with new-onset breast masses are recommended to undergo diagnostic mammograms and breast ultrasound for further evaluation. Those diagnosed with breast cancer most commonly have invasive ductal carcinoma of the breast, and over half of these patients are found to have estrogen and progesterone receptor (ER/PR) positivity. CASE REPORT In this case report, we present a Black man with gynecomastia and an areolar lesion for a 6-month duration following a traumatic event. He was initially referred to the surgical team for further evaluation, and subsequent imaging and biopsy data revealed ER/PR-positive invasive ductal carcinoma. Multidisciplinary discussions were held, and the patient was arranged to begin neoadjuvant treatment with doxorubicin hydrochloride and cyclophosphamide, followed by treatment with paclitaxel (AC-T) chemotherapy, followed by bilateral mastectomy and adjuvant hormonal therapy. CONCLUSIONS The treatment of male breast cancer has remained relatively like that of female breast cancer, which may be due to the limited data in the treatment of male breast cancer. Thus far, studies involving neoadjuvant chemotherapy of female patients have demonstrated promising responses to expand surgical options for patients and possibly decrease the rates of recurrence. Additional studies are warranted to discern optimal therapy for the male patient population.

An osteolytic lesion on imaging can be considered malignancy until proven otherwise. However, advanced stages of gout have presented with sclerotic rims and lytic lesions thought to be due to overexpression of osteoclasts. Patients have been found to demonstrate osteolytic lesions in patellar regions, which are common locations for gout to manifest; however, to our knowledge, no other cases of osteolytic gout in the acromioclavicular joint have been reported at this time. We report a rare case of a 56-year-old male who presented with acute-on-chronic left upper extremity pain and was found to have an osteolytic lesion of the shoulder on imaging. This lesion was later biopsied and found to be histologically consistent with gout. This case report aims to elucidate further understanding of the various ways that gout can present, to diagnose and treat these patients more effectively., (© 2022 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

SIADH is more commonly associated with small cell lung cancer but has also been associated with other malignancies such as oropharyngeal, gastrointestinal, genitourinary, endocrine, lymphomas, and sarcomas. There have been few reports of small cell carcinoma of the cervix complicated by SIADH; however, not many cases are associated with squamous cell carcinoma of the cervix. We present a case of a patient with squamous cell carcinoma of the cervix with a paraneoplastic syndrome of inappropriate secretion of antidiuretic hormone. The pathophysiology, clinical picture, and treatment are also discussed., Competing Interests: Conflict of Interest The authors report no conflict of interest. Ethical review is not necessary, because this is a case report. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors., (© 2022 Greater Baltimore Medical Center.)

FOLFIRINOX has been commonly utilized to treat patients with pancreatic cancer; however, it can manifest with rare, significant adverse effects. In particular, 5-FU has been associated with cardiotoxic effects, including but not limited to ischemic events, myocarditis, cardiac arrhythmias, cardiac death, heart failure, as well as coronary vasospasm. Two common thought processes regarding the mechanism of cardiotoxicity with 5-FU include exacerbation of ischemia secondary to coronary vasospasm and direct cell injury to the myocardium. Management of cardiotoxic adverse effects includes discontinuing 5-FU therapy if the patient can tolerate an alternative regimen or initiating prophylactic antianginal treatments with very close monitoring of the patient while they receive 5-FU therapy. Here, we describe a case of a 77-year-old patient with stage III pancreatic cancer who developed coronary vasospasm after initiation of combination therapy including 5-FU. Additional studies to gain further understanding of 5-FU cardiotoxicity are warranted, especially considering the common use of this medication with regards to pancreatic cancer patients. Further research of this topic may benefit patient care, prevent cardiovascular events, and determine which patients may benefit from prophylactic therapy while receiving 5-FU., Competing Interests: Conflict of interest The authors report no conflict of interest. Ethical review is not necessary, because this is a case report. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors., (© 2022 Greater Baltimore Medical Center.)

Cerebral venous sinus thrombosis (CVST) is a rather uncommon disorder. CVST is potentially lethal, therefore early detection and treatment is critical. CVST has been linked to pregnancy and puerperium, while COVID-19 infection has been linked to a hypercoagulable state. CVST can be difficult to detect and treat due to the wide range of neurological manifestations, especially in patients with hypercoagulability. The goal of this study is to conduct a literature review and present a unique case of a pregnant woman with CVST who had left hemiplegia and headache. After 6 months of treatment in the hospital, the patient's hemiplegia was fully resolved. Here, we discuss the treatment of CVST in pregnant women who have a suspected past COVID-19 infection., (© 2022 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Pulmonary carcinosarcoma (PCS) is a rare type of non-small cell cancer. Overall, middle-aged and older smokers are the most affected age and sex groups. The diagnosis of PCS is difficult due to the absence of characteristic imaging findings. Additionally, preoperative biopsies do not usually reflect the heterologous nature of this tumor. Given the rarity of such tumors and the challenging diagnosis, the prognostic factors have not been established, and the overall prognosis remains poor. The valid therapeutic options are still limited. Here, we report a rare case of metastatic PCS that was accidentally discovered by imaging and properly diagnosed after surgical resection. The clinicopathological features, diagnostic tools, genetic theories, prognosis, and therapeutic options of this rare cancer are also discussed., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Mekheal et al.)

Spindle cell carcinoma (SpCC)/sarcomatoid carcinoma is a biphasic tumor with molecular and histopathological properties of both epithelial and mesenchymal tumors. SpCC usually occurs either in sun-exposed areas like the head, neck, upper extremities, and chest or in the areas of skin with prior radiation exposure or in immuno-suppressed individuals. Cutaneous SpCC is a very rare disease, with only a handful of reported cases so far. SpCC differs from conventional squamous cell carcinoma (SCC) with dermal infiltration of atypical keratinocytes as single cells with hyperchromatic eosinophilic cytoplasm and elongated, pleomorphic nuclei with multiple nucleoli, in contrast to cohesive nests or islands in SCC. The objective of this study is to complete a review of the current literature and present a rare manifestation of malignant SpCC which developed from a localized basal cell carcinoma following excision and radiation therapy (RT) in a 79-year-old female. We plan to elucidate the importance of a timely and accurate diagnosis of this disease in order to maximize treatment options and improve survival outcomes., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Mekheal et al.)

Cancer patients are at a higher risk of complications such as venous thromboembolism (VTE). This risk increases in patients who receive chemotherapy. Despite the increased risk, common locations for VTE are similar to those in patients without cancer. Chemotherapy-port-related thrombosis represents a rare complication due to the location and frequent use of access (with Heparin flushes) as part of the standard care. Attention should be made to this rare complication, which may progress to superior vena cava (SVC) syndrome. SVC syndrome typically presents in females around the age of 57 years old. Management of this syndrome can be difficult and generally requires the initiation of systemic anticoagulation therapy. Here, we present a rare case of a 45-year-old female who presented to the Emergency Department with right arm swelling, found to be secondary to her chemo port thrombosis, causing SVC syndrome., Competing Interests: Conflicts of interest There is no conflict of interest., (© 2022 Greater Baltimore Medical Center.)

Nonbacterial thrombotic endocarditis (NBTE) also called, "Marantic endocarditis" occurs due to an underlying hypercoagulable state causing tissue damage and upregulation of the coagulation cascade, with noninfective vegetation formation on heart valves. Mitral and aortic valves are most commonly involved. NBTE is rare, with an incidence of 1.6%, with 65 cases identified during a 10-year autopsy analysis. The most common malignancies associated with NBTE include gynecological cancers, lung cancer, gastric cancer, and pancreatic cancers with adenocarcinoma histology being the greatest risk. Herein, we present a rare case of a 55-year-old male who presented with acute hypoxic respiratory failure secondary to pulmonary embolism due to nonbacterial thrombotic endocarditis. He was found to have advanced pancreatic adenocarcinoma on further investigation of the 2.2 cm hypodense cystic mass in the distal pancreatic body and tail, and complex liver masses which were incidentally found on computed tomography angiography (CTA) of the chest. This is a rare phenomenon and clinicians have to consider the hypercoagulable state associated with cancers, particularly pancreatic adenocarcinoma, and the risk of NBTE., (© 2022 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Adrenocortical cancer is a rare malignancy of the endocrine system. Therefore, when this malignancy presents with metastatic disease, this leads to further difficulties in management. Due to the rare and ambiguous nature of this malignancy, diagnosis is generally made at later stages, with limited options for patients. Symptoms may include weight gain/loss, muscle weakness, abdominal discomfort/bloating, hyperglycemia, hypertension, electrolyte imbalance, hirsutism and virilization in females, gynecomastia and hypogonadism in males. Due to the variety of conditions presenting with one or more of these symptoms, diagnosis can be difficult. Many adrenocortical tumors, malignant and benign, are usually detected incidentally on imaging performed for evaluation of another condition, also known as "incidentalomas." Here, we present a rare case of metastatic adrenocortical malignancy in a 56-year-old female patient who presented with isolated recurrent episodes of hypokalemia., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Mirza et al.)

Splenomegaly is manifested by a variety of etiologies, one of which is macrocytic anemia. Macrocytic anemia has multiple causes in itself that include; folate (Vitamin B9) and Cobalamin (vitamin B12) deficiencies. In this case report, we present a patient with a history of pancytopenia, macrocytic anemia and vitamin B12 deficiency, who underwent a splenectomy. The differential diagnoses for the cause of the patient's splenomegaly included: lymphoma, infiltrative disease, and idiopathic splenomegaly. The pathology report from the splenectomy did not reveal any evidence of lymphoma or infiltrative disease, however, it did mention vascular congestion of the spleen. In theory, vascular congestion, due to extramedullary hematopoiesis in the spleen or sequestration of blood cell lineages, could lead to pancytopenia. In prior visits to the hospital this patient was diagnosed with: splenomegaly, and macrocytic anemia due to pernicious anemia. A splenectomy puts one at increased risk for infection by encapsulated organisms, and is to be avoided if possible. There are few case reports and studies that show vitamin B12 therapy can potentially cause a reversal in the splenomegaly as well as a reversal in the pancytopenia and macrocytic anemia. We hope to show that the least invasive treatment for vitamin B12, vitamin therapy, can be of use and effective., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2022 Alexis Lordi et al.)

Follicular lymphoma is the most common type of low-grade non-Hodgkin lymphoma and the second most common type of lymphoma. Primary extranodal follicular lymphoma is rare compared with nodular follicular lymphoma involving the gastrointestinal (GI) tract. There has been uncertainty regarding follicular lymphomas due to the heterogeneous presentation and severity in which they present. However, studies showed that patients diagnosed with primary gastrointestinal follicular lymphoma do not typically differ in their presentation from those diagnosed with nodular follicular lymphoma involving the GI tract. Furthermore, recent literature identifies that patients diagnosed with grade 3 follicular lymphoma tend to have similar genetic and molecular entities to those diagnosed with diffuse large B-cell lymphoma (DLBCL). Based on these results, current studies have shown that patients with grade 3 follicular lymphoma who are treated with anthracycline-based regimens have similar outcomes to those with diffuse large B-cell lymphoma. However, additional studies are warranted to demonstrate the benefit of managing grade 3 follicular lymphoma with more aggressive anthracycline/rituximab-based regimens. Here, we present a case of a 44-year-old male diagnosed with grade 3 follicular lymphoma involving the gastrointestinal tract, who demonstrated an excellent treatment response following therapy similar to the treatment of bulky diffuse large B-cell lymphoma despite a tumor burden size below 7.5 cm., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Mekheal et al.)

Herein we present the case of a patient who was diagnosed with membranoproliferative glomerulonephritis and underwent renal transplant 27 years prior to presentation with new kidney failure. Although our patient did not undergo renal biopsy, it is our thought that she developed recurrent membranoproliferative disease, as she was well maintained on immunosuppressants and steroids for many years. This case is unique, because she was outside of the typical window for both chronic rejection and recurrent disease. This case also raises awareness of the utility of renal biopsy to differentiate these two conditions, which allows physicians to treat accordingly., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Tagliaferri et al.)

Complete blood counts are frequently collected from cancer patients, but laboratory findings may be misleading. Secondary polycythemia can occur in renal cell carcinoma (RCC) due to erythropoietin (EPO) stimulation. Therefore, complete blood counts should be closely monitored to prevent complications such as thrombosis. We discuss the case of a 47-year-old man with metastatic RCC who presented with secondary polycythemia that improved with chemotherapy. His secondary erythrocytosis was anticipated, but his haemoglobin levels were lower than expected after therapy. This article discusses the treatment and diagnosis of secondary polycythemia in patients with RCC., Learning Points: Haemoglobin and haematocrit levels should be closely monitored in renal cell carcinoma (RCC) patients as they may develop secondary polycythemia as a result of their malignancy.Secondary polycythemia can be managed with chemotherapy and immunotherapy in RCC, with anti-cancer agents preventing the need for phlebotomy.Chemotherapy may benefit RCC patients by decreasing tumour burden, preventing progression, and by lowering haemoglobin levels, thus improving secondary polycythemia., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests., (© EFIM 2022.)

Rosai-Dorfman disease (RDD) is a rare disorder characterized by the proliferation of histiocytes in lymph nodes. It can occur anywhere in the body but commonly involves the cervical area of the neck. Its clinical presentation varies and patients with skin manifestations may develop papules, nodules, plaques, or pustules. Histologically, it typically presents with emperipolesis, where intact lymphocytes are found within histiocytes. The definitive treatment of RDD is not well established given the rarity of the disease and indeed skin lesions can regress spontaneously. Therapeutic treatment options include cryotherapy, radiation, or topical agents such as steroids or retinoids. Here we describe the case of a 24-year-old Hispanic female who presented with skin manifestations which proved to be histologically positive for Rosai-Dorfman disease. The patient clinically improved following the administration of intralesional steroids., Learning Points: RDD is a rare self-limiting benign lymphoproliferative disorder, which can be associated with autoimmune disease, hematological malignancies, and post-infectious conditions.Skin lesions can be self-limiting in many cases of RDD.Therapeutic treatment options include cryotherapy, local radiation, and topical steroids and retinoids., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests., (© EFIM 2022.)

A 75-year-old male presented with lower back pain, bilateral lower extremity weakness, decreased sensation to vibration and proprioception in lower extremities, anemia, and vitamin B12 deficiency. The MRI of the lumbar spine revealed extensive leptomeningeal carcinomatosis. Subsequently, the patient was diagnosed with multiple myeloma (MM) and B12 deficiency with negative intrinsic factor antibodies. MM can present as extramedullary hematopoiesis (EM) to involve the central nervous system (CNS). CNS involvement is rare and develops in only around 1% of MM patients. It carries a poor prognosis with less than 6 months survival. MM is thought to be associated with both B12 deficiency and pernicious anemia. Some studies have even suggested B12 deficiency as a possible marker for worsening disease and a prognostic factor. In our patient's case, he had extensive CNS involvement at diagnosis of MM with very low B12 levels. The extent of his disease with extensive CNS involvement, which carries a poor prognosis, could possibly explain the very low levels of B12. This is the first reported case of a patient presenting with B12 deficiency found to have MM with leptomeningeal carcinomatosis at diagnosis. To the author's knowledge, there is no literature investigating association between B12 deficiency at the time of diagnosis of MM with CNS complications. Furthermore, there are no established guidelines on treatment for leptomeningeal myelomatosis. We present this case with the effort to learn more about this disease in terms of response and overall survival., Competing Interests: Conflict of interest All authors declare that there are no conflicts of interest., (© 2022 Greater Baltimore Medical Center.)

Patients with diabetes mellitus have diabetic dyslipidemia that occurs due to disturbances in glucose metabolism and can lead to hypertriglyceridemia (HPTG). Severe HPTG is associated with significantly increased risk of developing acute pancreatitis (AP). Acute pancreatitis (AP) is characterized as an inflammatory condition where inactive digestive enzymes become activated causing pancreatic tissue destruction. Hypertriglyceridemia and the inflammatory state that ensues therein also gives rise to a hypercoagulable state in patients with AP. Splenic vein thrombosis (SVT) is a rare complication of both AP and chronic pancreatitis (CP). We report a Case of 55-year-old Filipino male with past medical history of hypertension and uncontrolled type 2 diabetes mellitus (T2D), who presented with abdominal pain and was found to have diabetic ketoacidosis (DKA), and severe HPTG which led to acute pancreatitis, further complicated by SVT requiring anticoagulation. Our case highlights the importance of strict glycemic control among diabetic patients, the prompt management of AP in the setting of HPTG, and treatment of SVT., Competing Interests: Conflict of interest All authors declare that there are no conflicts of interest., (© 2022 Greater Baltimore Medical Center.)

Acute promyelocytic leukemia (APL) is a unique subtype of acute myeloid leukemia (AML), which presents with a distinct coagulopathy. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia which is clonal in nature due to somatic mutation. PNH may evolve to aplastic anemia, and more rarely, to a myelodysplastic syndrome or to AML. The literature review showed that AML is derived from the PNH clone as the leukemic cells lack the expression of glycosylphosphatidylinositol-linked proteins and PNH phenotype disappeared with the onset of acute leukemia. Herein, we report an unusual presentation of the coexistence of two clonal disorders PNH and APL. Our case contributes to the literature that AML in the setting of PNH is a separate disorder., Competing Interests: None

Patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection mainly present with upper and lower respiratory tract symptoms, with complications related to cytokine storm syndrome and acute respiratory distress syndrome. It has also been described to predispose to venous and arterial thromboembolism; however, limited published data is available regarding thrombosis in coronavirus disease 2019 (COVID-19). Here we are presenting a case of arterial thrombosis in a patient with COVID-19 and a systematic review on coagulopathy associated with COVID-19., (© 2020 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd.)

Although cytomegalovirus (CMV) encephalitis is a common viral infection, it is rarely reported in immunocompromised patients with cluster of differentiation four (CD4) cell counts greater than 50. Herein, we present a case of CMV encephalitis co-infected with Epstein-Barr virus (EBV) in a human immunodeficiency virus (HIV) patient with a CD4 cell count of 145. In addition, the patient was also infected with syphilis and tuberculosis. This case report will discuss the complications of untangling the differential diagnosis in an immunocompromised host with multiple infections, specifically, how it was difficult to identify the exact etiology of this patient's encephalopathy. We will address the plausible explanations for this unusual presentation, including CD4 dysfunction, latent and re-infections, and synergism seen with the co-infections in HIV patients., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Narvaneni et al.)

Competing Interests: None of the authors have conflicts of interest.

Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis (LCH) that affects different body systems. It was recently recognized as a neoplastic disorder after identifying an activating mutation of the MAPK pathway. Neurological presentations of ECD are rare. We present a case of a 35-year-old male who presented to the emergency department with neck pain, headache and vomiting for 2 months; MRI showed multiple heterogeneous intracranial masses. Neurosurgery performed a suboccipital craniotomy, partially resected the cerebellar mass, and placed a parietal to frontal shunt catheter. Biopsy results from the cerebellar mass demonstrated cerebellar tissue involved by a diffuse proliferation of foamy histiocytes and spindle cells admixed with prominent lymphoplasmacytic infiltrate and positive for CD68, CD163, Factor XIIIa and Fascin. PET scan showed hypermetabolic uptake within the medullary portions of the diffuse abnormal lesions of the distal femurs, tibias, and fibulas, and cardiac MRI was nonsignificant. The patient was started on vemurafenib and continued to show improvement in a 3-month outpatient follow-up., Competing Interests: Conflict of interest: The authors have no conflict of interest to declare., (Copyright © 2021 The Author(s).)

The SARS-CoV-2 infection has been predominately associated with lung disease. However, emerging evidence has associated the COVID-19 infection with a hypercoagulable state. This hypercoagulable state can occur despite the use of anticoagulants and antiplatelets. In fact, it may even be the presenting symptom of COVID-19 in some patients. Thromboembolism associated with COVID-19 carries a worse prognosis and should be identified as early as possible. Therefore, we report 2 patients with arterial thrombosis in the form of limb ischemia in the setting of COVID-19., (© 2021 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has caused the ongoing global pandemic. It can manifest a wide range of complications depending upon the severity of infection and comorbidities of the patient. Vaccines are very important measure to provide protection against COVID-19. We report a case of 72-year-old female with past medical history of hypertension and diabetes mellitus who underwent imaging with positron emission tomography (PET) scan imaging for staging of her small cell urinary bladder cancer and was found to have hypermetabolic uptake in the deltoid muscle of the left shoulder and hypermetabolic left axillary and pectoral lymph nodes due to mRNA BNT-162b2 (Pfizer-BioNTech COVID-19 vaccine) vaccine administrated 3 days ago prior to PET scan., (© 2021 Published by Elsevier Inc. on behalf of University of Washington.)

Clozapine has been widely used as an antipsychotic drug for the treatment of refractory schizophrenia. Unfortunately, a wide range of blood dyscrasias have been reported as adverse effects of this drug. Agranulocytosis has gotten the most clinical vigilance; however, there are substantial reports of other blood dyscrasias associated with Clozapine some more serious than others. Of relevance, there have been previous claims of Clozapine-associated leukocytosis and acute myeloid leukemia. We report the case of a 31-year-old patient who developed Acute lymphoblastic leukemia shortly after starting treatment with Clozapine for refractory schizophrenia. We suggest Clozapine may play a causal role in the development of leukemias in patients taking this medication and we encourage vigilance for such correlation., (© 2021 The Author(s). Published by Elsevier Ltd.)

Acquired Hemophagocytic Lymphohistiocytosis is a rare and deadly syndrome resulting from an overactive immune system, with uncontrolled activation of macrophages and lymphocytes, hypercytokinemia, and systemic inflammatory response. A 75-year-old male presented with typical anginal pain and was diagnosed with the acute coronary syndrome, which required a percutaneous transluminal coronary angioplasty. Instead of resolving the symptoms, the patient began to exhibit pyrexia and worsening altered sensorium with progressing renal failure, anemia, thrombocytopenia and respiratory failure. This constellation of symptoms caused the patient to require mechanical ventilation and hemodialysis. Upon laboratory analysis, hyperferritinemia provided an indication to the diagnosis of acquired hemophagocytic lymphohistiocytosis. After the initiation of dexamethasone, the patient made a significant recovery and was discharged from the hospital., Competing Interests: Conflict of interest: The authors declare that they have no conflicts of interest., (Copyright: © 2021 The Authors.)

Aim To describe the clinical characteristics and outcome of hospitalized COVID-19 patients with diabetic ketoacidosis (DKA). Methods We report eight cases of diabetic ketoacidosis in COVID-19 who presented to our institution in New Jersey, USA. COVID-19 was diagnosed by nasopharyngeal swab reverse transcription polymerase chain reaction (RT-PCR). The patients' electronic medical records were reviewed. Data on patients' age, sex, ethnicity, laboratory values, glycosylated hemoglobin level, oral antihyperglycemic agents (OHAs), insulin, and clinical outcomes were collected. Results The median age of the patient was 42.5 years, and seven were males and one was female. Out of eight patients, five had type 2 diabetes mellitus (DM), two had undiagnosed DM, and one had type 1 DM. Median value of initial glucose on presentation was 454 mg/dL. Median value of HbA1c on presentation was 11.4% and of anion gap was 26.5 mEq/L. Four patients had large ketonemia, one patient had moderate ketonemia, and three patients had small ketonemia. All the patients were started on standard treatment protocol for DKA with intravenous fluids and IV insulin infusion. Acute kidney injury (AKI) was seen in four patients, and one patient required renal replacement therapy. Out of eight patients, three required mechanical ventilation, and the same three patients died. Conclusion Our case series shows that COVID-19 infection can precipitate DKA in patients with known diabetes mellitus patients or as a first manifestation in undiagnosed DM patients; COVID-19 with DKA is associated with substantial mortality. Further studies are needed to characterize poor risk factors associated with mortality in these patients., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Singh et al.)

Cannonball lesions are numerous, well-circumscribed, round pulmonary lesions and may be identified on plain radiograph or advanced imaging. This morphology can be associated with infectious causes, rheumatologic disease and metastatic disease. Classically, when cannonball lesions are associated with metastatic disease, they are seen in renal cell carcinoma and choriocarcinoma. We report a case of a 62-year-old Middle Eastern male who presented with shortness of breath, chest pain and fatigue and was found to have bilateral multiple pulmonary cannonball lesions and mass in the pancreas. Biopsy of one the lung lesions was consistent with pancreatic cancer. Our case adds to the limited literature available regarding cannonball lung lesions in the setting of pancreatic cancer. Health care providers should be aware of the various etiologies of cannonball lesions., (© 2021 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a novel coronavirus responsible for the current global pandemic, coronavirus disease 2019 (COVID-19). COVID-19 usually presents with respiratory symptoms but can affect multiple organ systems. A wide spectrum of complications can occur depending upon the comorbidities of patients. There is limited literature available regarding the presentation and outcome of COVID-19 in chronic lymphocytic leukaemia (CLL) patients. We report 2 cases of COVID-19-induced hyperleucocytosis (WBC count >100,000/μl) in CLL patients., Learning Points: Lymphopenia has been associated with severe disease and is a poor prognostic factor in COVID-19 infected patients; however, our cases show COVID-19-induced hyperleucocytosis (WBC count >100,000/μl)/lymphocytosis in CLL patients.Prior reports suggest that ibrutinib may have a protective effect against COVID-19 by decreasing inflammation and preventing progression to ARDS., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests., (© EFIM 2021.)