High-Grade B-Cell Lymphoma (HGBCL) with gene rearrangements in MYC and BCL2 and/or BCL6 is an aggressive malignancy usually presenting in advanced stages. Current recommendations suggest the use of regimens more intensive than R-CHOP (rituximab, cyclophosphamide, vincristine, doxorubicin, prednisone), which are based on retrospective studies and single-arm prospective trials that included patients who are mostly in the advanced stage, and did not receive consolidation radiotherapy.

The optimal approach and treatment of HGBCL, whether limited-stage (LS) or advanced-stage, remains to be determined. Here we describe the promising outcomes of three patients with LS and low IPI HGBCL with the use of R-CHOP as induction chemotherapy regimen, which was followed by consolidation radiotherapy.

Three women, 54-, 60-, and 64-years of age diagnosed to have HGBCL with MYC, and BCL2 and/or BCL6 rearrangements, with Ann Arbor stages I-IIE were included in this case series. All three patients had complete metabolic response to 6 cycles of R-CHOP and was subsequently treated with consolidation involved site radiotherapy (ISRT; total dose 30-36 Gy). Chemotherapy and radiotherapy were tolerated very well. All patients remain to be in remission, with the longest being at 23 months.

Outcomes of patients with HGBCL generally remain to be poor, but this may not be the case for patients with limited-stage disease and favorable clinicopathologic risk profile. Nevertheless, the treatment of HGBCL is currently evolving and more studies are needed to determine the ideal approach and preferred chemotherapy regimen. Also, more studies are needed to elucidate the potential role of consolidation radiotherapy in patients with limited-stage HGBCL to improve survival outcomes. Findings of this case series suggest that patients with LS HGBCL may still derive benefit from R-CHOP followed by consolidation ISRT, but prospective trials are needed to confirm this.

BACKGROUND

Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL). Classification of DLBCL is often based on the cell of origin (COO), distinguishing between germinal center B-cell (GCB) and non-GCB subtypes. Although not yet recognized as a distinct entity by the World Health Organization (WHO), double expressor lymphoma (DEL), characterized by the co-expression of c-MYC and BCL2, carries an unfavorable prognosis for a subgroup of DLBCL patients. Another entity is the so-called high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements (double-hit/triple-hit lymphomas) diagnosed through fluorescent in-situ hybridization (FISH) analysis.

This study aimed to determine the clinicopathologic profile and survival outcomes of Filipino DLBCL patients at the Philippine General Hospital (2016-2021), comparing double-hit versus non-double-hit and doubleexpressor versus non-double-expressor lymphomas, and assessing concordance between FISH-measured double-hit and IHC-measured double-expressor statuses.

This is a single-arm, retrospective cohort study involving all surgical pathology cases signed out, with the aid of immunohistochemistry (IHC) studies, as NHL DLBCL, GCB, or non-GCB subtype, from 2016 to 2021. A second panel of IHC studies and FISH analysis using tissue microarray was subsequently done. Most cases exhibited a nonGCB subtype and were classified as DEL on second IHC panel. Five out of eleven DEL cases were reclassified as double hit lymphoma (DHL).

Clinically, most patients with these lymphomas present at age 60 years and below, exhibit B symptoms, with elevated serum lactate dehydrogenase (LDH) levels, at least stage III-IV disease at diagnosis, and possess a high International Prognostic Index (IPI) score, collectively indicating a poor prognosis.

Survival outcomes for patients with DLBCL ranges from three to 37 months. All cases of mortality were associated with DEL, contrasting with DHL cases which had variable outcomes. Due to limited sampling, statistical significance of the results cannot be determined. A comprehensive evaluation is essential to the diagnosis of DLBCL and DHL to include a complete immunohistochemistry panel and molecular testing, notably with FISH studies.

INTRODUCTION

Parotid lymphoma is a rare occurrence, let alone a diagnosis of nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). Salivary gland tumors commonly affect the parotid gland, although a primary malignant lesion rarely occurs, with an incidence of 0.5 to 3.0/100,000 population/year worldwide. This case report describes the presentation of this rare lymphoma. This also demonstrates the efficacy of standard of care chemotherapy with doxorubicin, vincristine, bleomycin, and dacarbazine with an anti-CD20 monoclonal antibody, rituximab (R-ABVD).

This is a case of a 44-year-old male with a gradually enlarging right preauricular mass. Biopsy and immunohistochemical staining confirmed a diagnosis of NLPHL Stage IIA. A total of six cycles of chemotherapy with R-ABVD was given. Follow-up PET CT showed resolution of FDG avid nodes localized near the surgically removed parotid gland, confirming complete remission.

Parotid malignancy only accounts for 5% of all head and neck tumors. NLPHL is even more rare, with an incidence of 1.5/1,000,000 population per year. The rarity of the case limits clinical trials for its treatment. Because of this, R-ABVD has been employed as a treatment of choice for intermediate-staged NLPHL. Overall response showed an 85% five-year progression-free survival and 99% overall survival.

This case report highlights the significance of early lymphoma detection despite its rarity among parotid tumors and prompt initiation of chemotherapy.

Background: Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL). Classification of DLBCL is often based on the cell of origin (COO), distinguishing between germinal center B-cell (GCB) and non-GCB subtypes. Although not yet recognized as a distinct entity by the World Health Organization (WHO), double expressor lymphoma (DEL), characterized by the co-expression of c-MYC and BCL2, carries an unfavorable prognosis for a subgroup of DLBCL patients. Another entity is the so-called high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements (double-hit/triple-hit lymphomas) diagnosed through fluorescent in-situ hybridization (FISH) analysis. Objective: This study aimed to determine the clinicopathologic profile and survival outcomes of Filipino DLBCL patients at the Philippine General Hospital (2016-2021), comparing double-hit versus non-double-hit and doubleexpressor versus non-double-expressor lymphomas, and assessing concordance between FISH-measured double-hit and IHC-measured double-expressor statuses. Methods: This is a single-arm, retrospective cohort study involving all surgical pathology cases signed out, with the aid of immunohistochemistry (IHC) studies, as NHL DLBCL, GCB, or non-GCB subtype, from 2016 to 2021. A second panel of IHC studies and FISH analysis using tissue microarray was subsequently done. Most cases exhibited a nonGCB subtype and were classified as DEL on second IHC panel. Five out of eleven DEL cases were reclassified as double hit lymphoma (DHL). Results: Clinically, most patients with these lymphomas present at age 60 years and below, exhibit B symptoms, with elevated serum lactate dehydrogenase (LDH) levels, at least stage III-IV disease at diagnosis, and possess a high International Prognostic Index (IPI) score, collectively indicating a poor prognosis. Conclusion Survival outcomes for patients with DLBCL ranges from three to 37 months. All cases of mortality were associated with DEL, contrasting with DHL cases which had variable outcomes. Due to limited sampling, statistical significance of the results cannot be determined. A comprehensive evaluation is essential to the diagnosis of DLBCL and DHL to include a complete immunohistochemistry panel and molecular testing, notably with FISH studies.
lymphoma ; lymphoma, large B-cell, diffuse ; cytogenetics ; immunohistochemistry

Lymphomatoid Papulosis (LyP) is a rare skin disorder characterized by chronic, recurrent papules and nodules that heal spontaneously. This report discusses an 8-year-old Filipino female with a three-month history of erythematous pruritic papules evolving into plaques. A skin biopsy confirmed CD30-negative LyP, and treatment with Methotrexate resulted in significant improvement of the lesions. This case illustrates the complex diagnostic and therapeutic journey of LyP in children, emphasizing the importance of careful clinicopathologic correlation and the challenges of management due to the lack of curative therapy and the risk of malignant transformation.

For diagnosis of rare conditions, consistent follow-up on the part of the patient as well as a high index of suspicion on the part of the physician is needed. Evaluation of the management should be done in the event that patients do not respond to treatment.

This is a case of a 33 year old female who fifteen months prior, noticed erythematous scaly plaques on her cheeks with mild pruritus. She was treated for psoriasis with Halobetasol ointment and Petroleum Jelly, which had partial resolution. Twelve months prior, she was prescribed Methotrexate, again achieving partial resolution. Biopsy was done suggestive of Folliculotropic Mycosis Fungoides. Methotrexate was increased, and additional medications were prescribed, leading to lesion resolution. However, she was lost to follow-up and experienced worsening symptoms. One month prior, biopsy was repeated and again showed Diffuse Lymphocytic Dermatitis positive for CD3+, CD4+, CD5+, CD8-, CD20-, CD30-, and loss of staining for Pan-T cell markers, CD2- and CD7. She is currently managed with regular sessions of Narrowband UVB.

These rare cases are few but more often than not, they are easily missed and when caught are usually progressed and already difficult to treat. Physicians must be vigilant in treating patients, even if they initially diagnose it to be a commonly seen and easily managed disease. Skin Directed therapy is done with PUVA and NBUVB with complete response in 30-70%3. For prognosis, early stages are favorable with a 94% 5 year survival rate, decreasing to 69% after tumor development.

18F-Fluorodeoxyglucose (18F-FDG) PET/CT scan is a vital imaging modality in the majority of oncologic situations. It is proven useful in staging, management and monitoring of lymphomas. Numerous subtypes of lymphomas exist; however, we present the first documented case of a 56-year-old, Filipino, male patient who is diagnosed with mantle cell lymphoma of the conjunctiva (MCL). MCL is an extremely rare type of extranodal non-Hodgkin lymphoma and has an aggressive nature with an estimated incidence of 2-4/1,000,000. This case highlights the critical role that PET/CT scans play in directing treatment decisions and monitoring the response of conjunctival MCL to therapy.
Lymphoma, Non-hodgkin ; Positron-emission Tomography

Constitutionalmismatch repair deficiency(CMMRD) is a hereditary predisposition of malignancy evident in childhood leukemias, lymphomas, and malignant tumors of the brain, GI tract. It is a very rare condition that affects 1 per 1 million patients. Patients with CMMRD syndrome may also manifest with Neurofibromatosis Type 1 (NF1) phenotypic features, and benign masses, particularly in the gastrointestinal tract. This is a case of a 12-year old male who presented with phenotypic features of NF1, developed Acute Lymphoblastic Leukemia at 7 years old and went into remission. He subsequently developed synchronous Glioblastoma and Poorly differentiated Adenocarcinoma of the rectum.This report aims to raise awareness regarding the possibility of a CMMRD syndrome in pediatric patients who present with phenotypic features of NF1, and in those patients who present with two or more malignancies in their lifetime.
Glioblastoma ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

Humans ; HIV-1 ; Leukocytes, Mononuclear ; Lymphoma, Non-Hodgkin ; DNA ; HIV Infections

Objective: To summarize the therapeutic effects of Chinese Children Leukemia Group-acute lymphoblastic leukemia (CCLG-ALL) 2018 regimen in children with T cell acute lymphoblastic leukemia (T-ALL) and to find out risk indicators for prognosis. Methods: This study was a prospective multicenter cohort study involving 299 newly diagnosed T-ALL children in 21 Grade A tertiary hospitals nationwide. All patients received CCLG-ALL 2018 regimen and clinical data for treatment efficacy evaluating was collected. Variables associated with event free survival (EFS) rate, overall survival (OS) rate and cumulative recurrence rate were evaluated by Lasso regression analysis (including variables selection, model construction and hazard ratio calculating). Results: A total of 299 newly diagnosed T-ALL children were included, accounting for 9.9% (299/3 026) of all ALL patients. Among these patients, there were 224 males and 75 females, and the age of onset was 7.0 (4.7, 10.6) years. All patients received CCLG-ALL 2018 regimen treatment. After 31.1 (17.3, 43.8) months follow-up, 3-year EFS, 3-year OS and cumulative recurrence rate of them were (83.2±2.7)%, (91.3±1.8)%, and (7.9±1.7)%, respectively. Minimal residual disease (MRD) greater than 10.00% on day 15 of induction therapy was a risk factor for EFS (HR=1.89, 95%CI 1.04-3.44), OS (HR=2.82, 95%CI 1.35-5.92), and cumulative recurrence rate (HR=3.05, 95%CI 1.46-6.34). Compared with the medium-risk group, the high-risk group had higher induction failure rate (5.2% (7/134) vs. 0 (0/145), P=0.016) and lower complete remission rate (88.8% (119/134) vs.97.9% (142/145),P=0.004). Most complications happened during induction therapy (95 cases), and the most common complication was serious infection (158 cases). Conclusions: CCLG-ALL 2018 regimen shows good prognosis. MRD greater than 10.00% on day 15 of induction therapy is a strong risk factor, which can indicate the prognosis in the early stage of the disease and guide the appropriate treatment.
Male ; Female ; Humans ; Child ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/drug therapy* ; Disease-Free Survival ; Prospective Studies ; Cohort Studies ; East Asian People ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Treatment Outcome ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy* ; Prognosis ; Neoplasm, Residual