Approach to Gender Affirming Care in a Patient with Sporadic High Risk Burkitt Lymphoma

M. Abbasi¹, C. Leatheng¹, M. Stolpman²

1- Dartmouth Hitchcock Medical Center

2- Geisel School of Medicine

Background

There are no formal guidelines for Gender Affirming Care in Gender-Diverse/Transgender patients with cancer. They require a multidisciplinary approach, which includes creating a gender-affirming environment, psychosocial support and possible postponement of gender-affirming treatments (GAT).

Methods

A 37-year-old Affirmed Female (MTF), assigned male at birth, with recent diagnosis of Cavernous Sinus Thrombosis, presenting with new onset leg weakness with urinary retention, concerning for cauda equina syndrome. The patient was previously on GAT comprising of Spironolactone, Progesterone and Estrogen, which they started 5 years ago and had stopped 8 weeks prior to presentation, citing back pain. MRI Spine near-complete dura involvement from C6 to L1 causing severe cord compression. Lymph node biopsy resulted CD10+, BCL6+ BCL2- ; Ki-67 near 100%, +(8;14); 90-100% bone marrow involvement. She was started on high dose dexamethasone, Modified Magrath regimen with Rituximab (CODOX-M + IVAC + R), with intrathecal Cytarabine/Methotrexate and radiation therapy. Given her thrombosis, GAHT were held. Finasteride and GnRH agonists were considered, however held given multiple clinical complications, including cecal hematoma requiring an open colectomy.

Results

Our patient had high thrombosis risk given both Estrogen and Progesterone use, malignancy, smoking history and inability to initiate anticoagulation given GI bleeding. A case report on MTF patient with DLBCL stated discontinuing GAHT given Erβ association with DLBCL. Along with holding GAHT, patients commonly experience being misgendered which strain therapeutic relationships. Clinicians have indicated discomfort managing GDT patients due to lack of knowledge.

Conclusions

A priority of creating a gender-affirming environment should be made, which necessitates a multi-disciplinary approach.

Immune checkpoint inhibitor-induced myocarditis: a case report and considerations for management

A. Gates¹, C. Tretter²

1- MaineHealth Maine Medical Center Internal Medicine Residency

2- MaineHealth Cancer Care                        

Background

Immune checkpoint inhibitor-induced myocarditis is a rare but serious complication of immunotherapy. Current guidelines for immune-related adverse events lack specificity for this potentially life-threatening condition (NCCN, SITC, ASCO). This is a case of immune checkpoint inhibitor-induced myocarditis which exemplifies the need for more nuanced recommendations.

Methods

An 80-year-old male with Bacille Calmette-Guerin/gemcitabine unresponsive, high-grade superficial urothelial carcinoma completed one cycle of pembrolizumab and then experienced progressive dyspnea and diplopia, found to have grade four myocarditis, myositis, and myasthenia gravis. Medical oncology, cardiology, and neurology services closely followed his treatment course. He received several escalating immunosuppressive treatment modalities including steroids, intravenous immunoglobulin, plasma exchange, mycophenolate, colchicine, and ruxolitinib with little improvement in symptoms or cardiac biomarkers. He finally had improvement after an initial dose of abatacept.

Results

He stabilized and was ultimately given five additional doses of abatacept in conjunction with ruxolitinib (Salem) in the outpatient setting with continued improvement in symptoms and troponin.  He remains with moderate, stable troponin elevation 13 months after pembrolizumab infusion.

Conclusions: Patients with checkpoint-inhibitor induced myocarditis are often critically ill and can remain with lingering signs and symptoms of toxicity, just as this patient has experienced (Nielsen, Johnson). Therefore, we must be vigilant for side effects of immunotherapy with emphasis on prompt recognition, involvement of appropriate subspecialities, and initial treatment with high-dose steroids. Ongoing clinical trials may direct future recommendations for early initiation of additional therapies such as abatacept (ACHLYS, ATRIUM).

Relapsed B-cell Acute Lymphoblastic Leukemia Presenting as Alopecia and Peripheral Neuropathy: An Unusual Extramedullary Pattern in the Era of Targeted Therapy

A. Khamechand¹, A. Sharma¹, S. PonnamReddy¹

1- Dartmouth Hitchcock Medical Center

Background

Relapse of B-cell acute lymphoblastic leukemia (B-ALL) typically involves the bone marrow or central nervous system. However, emerging therapies such as anti-CD19 immunotherapy may influence relapse patterns, potentially increasing the frequency of atypical extramedullary presentations.

Methods 

Case Presentation

Results

Case Presentation:

We report the case of a 32-year-old man with B-ALL initially treated with the CALGB 10403 adolescent and young adult protocol, followed by blinatumomab and mismatched unrelated donor hematopoietic stem cell transplantation. He achieved minimal residual disease (MRD)-negative remission with >95% donor chimerism. Eighteen months post-transplant, he developed progressive right-sided peripheral neuropathy and alopecia. Imaging revealed FDG-avid scalp lesions and diffuse involvement of peripheral nerves and lymph nodes. Biopsy of the scalp lesion confirmed relapsed B-ALL. He was treated with R-Hyper-CVAD (arm B), achieving MRD-negative remission, and subsequently underwent CD19-directed CAR T-cell therapy (Tecartus). His course was complicated by grade 2 ICANS, which resolved with immunosuppressive therapy. Follow-up imaging demonstrated no evidence of disease.

Conclusions

This case highlights an exceedingly rare presentation of B-ALL relapse manifesting as alopecia and peripheral neuropathy—features not previously reported in the literature. It underscores the diagnostic challenge of extramedullary relapse in the era of targeted immunotherapy, where sanctuary sites may evade treatment. This case supports expanding surveillance strategies and considering broader imaging in patients with atypical symptoms post-treatment. CAR T-cell therapy remains a promising treatment option, even in cases of rare extramedullary recurrence.

T-VEC for Advanced Cutaneous Melanoma: A Case Series

H. Kuznia¹, K. Shirai¹

1- Dartmouth Hitchcock Medical Center

Background

Talimogene laherparepvec (T-VEC) is a modified herpes simplex virus (HSV-1) approved for the treatment of cutaneous melanoma. It selectively replicates in cancer cells and induces cell death, as well as enhances the immune response toward tumor cells. T-VEC is injected directly into tumor lesions for local effect, but has also shown effect on un-injected sites. Some hypothesize that T-VEC could enhance efficacy of immune-checkpoint inhibitor (ICI) therapies by increasing IFN-1 production which subsequently leads to increased immune inhibitory surface receptors like PD-L1. Studies exploring the effect of T-VEC combined with ICI therapy are ongoing, and thus far have shown limited significant findings.

Methods

We present a case series of seven patients with advanced cutaneous melanoma who received treatment with T-VEC.

Results

Two patients in this case series were responsive to rechallenge with T-VEC. All five other patients had clinical response to initial T-VEC. All seven patients received at least one dose of ICI therapy prior to their first T-VEC dose. Five of the patients developed vitiligo, which is thought to be a sign of immune responsiveness, and thus can indicate the efficacy of an immune therapy.

Conclusions

The use of T-VEC in conjunction and/or alternation with ICIs can lead to significant impact on melanoma disease control. Further studies clarifying this impact and chronicity of combination therapies are needed.  Oncolytic viruses such as T-VEC are an important tool in the oncologist’s toolbelt when managing advanced melanoma.

Improving Care Delivery for Rural New England Patients Receiving T-cell Engager (BiTE) Therapy: Project Charter and Preliminary Results

H. Kuznia1, Y. Soo Rho, C. Gaulin, O. Agogbuo, K. Karkowski, A. Houlne, A. Sharma

Dartmouth Hitchcock Medical Center

Background

Bispecific T-cell engager (BiTE) therapies are cancer therapies used for treatment of both hematologic and solid tumors. They require careful monitoring for significant adverse effects including cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS). At Dartmouth Hitchcock Medical Center (DHMC), the lack of standardization in BiTE workflows can predispose to suboptimal use of hospital resources, transitions of care, and patient outcomes.

Methods

For this quality improvement project, we developed a multidisciplinary team and created a two phase plan. Phase one includes assessment of current workflows, identification and survey of stakeholders, development of a standard operating procedure, and modification of current CRS and ICANS guidelines. Phase two includes reassessment of the workflow after phase one, creation of standardized documentation, implementation of outpatient workflows, and expansion to satellite sites.

Results

The phase one stakeholder survey compiled responses from 86 participants, with subgroups as follows:  RNs 33.7%, attending MDs 11.6%, APPs 5.9%, Fellow/Trainees 18.7%, Pharmacy 23.3%, and other 7%. Nearly half of those surveyed were familiar with the current workflows for BiTE for solid and hematologic malignancies (43% and 49%, respectively). Satisfaction rates with these workflows were more varied (20% in solid and 44% in hematologic). Specific concerns related to the BiTE workflows included space limitations, transitions of care, and standardization of monitoring duration as well as toxicity management.

Conclusions

The safe and effective administration of BiTE therapy is challenging in a setting where so many patients live rurally. We continue to focus on workflow improvement and standardization in delivery of BiTE therapy.

HPV Associated Nasopharyngeal Carcinoma

J. O'Farrell¹, G. Wasp² 

1- Dartmouth-Hitchcock Medical Center

2- Dartmouth Cancer Center

Background

This case describes the unique presentation of an HPV-associated carcinoma of nasopharyngeal, highlighting the importance of diagnostic accuracy in head and neck squamous cell cancer. This case acts to bring awareness to an atypical presentation of malignancy with a significant impact on the recommended treatment regimen.

Methods

The patient is a 63-year-old female who was evaluated by her primary care physician for a history of nasal blockage due to a nasopharyngeal mass. Biopsy was obtained and was shown to be p16 positive SCC, indicating an HPV associated malignancy. Given relation between HPV and oropharyngeal cancers, suspicion was raised about the true origin, whether it is nasopharyngeal or oropharyngeal. Patient was evaluated at DHMC for a second opinion where her diagnosis was confirmed as an HPV associated head and neck squamous cell carcinoma originating from the nasopharynx.

Results

In viral-associated head and neck malignancies, typically HPV is associated with malignancy originating in the oropharynx while EBV is more typically associated with malignancy originating in the nasopharynx. The importance of accurate diagnosis is highlighted in treatment choice. For most nasopharyngeal carcinomas what are associated with EBV, concurrent chemoradiation, induction chemotherapy, and adjuvant chemotherapy are utilized with curative intent.

Conclusions

This case illustrates the benefits of medical second opinions and diagnostic accuracy specifically in viral-associated head and neck squamous cell carcinomas. Although time consuming, a complete workup and evaluation of a clinical case by a tumor board can have a significant impact on treatment course and outcomes.

IgA Kappa Myeloma with Plasmablastic Morphology and Extensive Extramedullary Disease: A Rare Diagnostic and Therapeutic Challenge

V. Osadchyi¹, M. Dailey²

1- University of Connecticut Internal Medicine Residency

2- Hartford HealthCare Cancer Institute

Background

IgA Kappa multiple myeloma with plasmablastic histologic features and extensive extramedullary disease is a rare and aggressive plasma cell disorder. These cases often exhibit an atypical clinical course with soft tissue and visceral involvement. Differentiating plasmablastic multiple myeloma from plasmablastic lymphoma is essential, as treatment strategies differ significantly. Comprehensive immunophenotyping is crucial for accurate diagnosis and optimal therapy.

Methods

A 57-year-old man with a history of medullary plasmacytoma treated with radiotherapy presented with progressive systemic symptoms concerning for disease dissemination. PET/CT revealed hypermetabolic extramedullary lesions, including in the retrocrural region, stomach, pleural space, abdominal wall, and skeletal system. Upper endoscopy identified a large, bleeding gastric mass. Biopsies underwent histopathologic and immunohistochemical analysis.

Results

Pathology demonstrated sheets of plasmacytoid cells positive for CD38, MUM1, and IgA-Kappa, consistent with multiple myeloma. Negative staining for CD20, PAX5, CD138, ALK, HHV8, and EBER excluded plasmablastic lymphoma. The patient received Dara-CyBorD (daratumumab, cyclophosphamide, bortezomib, dexamethasone) and completed five cycles. Follow-up PET/CT demonstrated near-complete resolution of FDG-avid lesions. He is now being evaluated for autologous stem cell transplantation.

Conclusions

This case highlights the importance of distinguishing plasmablastic myeloma from lymphoma for appropriate management. The patient’s response to Dara-CyBorD supports the role of CD38 monoclonal antibodies in high-risk myeloma with extensive extramedullary disease. Early diagnosis and targeted therapy are crucial for improving outcomes.