Effect of Pre-Treatment with a Combination of Fentanyl and Midazolam for Prevention of Etomidate-Induced Myoclonus.

OBJECTIVE: Pre-treatment with either fentanyl or midazolam has previously been used to prevent etomidate-induced myoclonus (EIM). The aim of the present study was to determine the effect of pre-treatment with a combination of midazolam and fentanyl in reducing the incidence and severity of EIM. METHODS: This prospective, randomised, double-blind study was conducted on 210 surgical patients allocated to three study groups. Group F patients received fentanyl 2 µg kg-1 and 5 mL saline. Group M patients received midazolam 0.03 mg kg-1 and 5 mL saline. Group FM patients received fentanyl 2 µg kg-1 plus midazolam 0.03 mg kg-1. The study drugs were administered intravenously over 30 s. Five minutes after study drug administration, etomidate 0.3 mg kg-1 was administered over 60 s. Patients were observed for 1 min for occurrence and severity of EIM. RESULTS: The incidence of EIM was 34/70 (48.6%), 55/70 (78.6%) and 11/70 (15.7%) in groups F, M and FM, respectively (p=0.001). Myoclonus of moderate or severe grade occurred in 23/70 (32.9%), 45/70 (64.3%) and 6/70 (8.6%) in groups F, M and FM, respectively (p=0.001). Patients who experienced myoclonus exhibited a significantly higher percentage change in post-induction heart rate (p=0.02), systolic blood pressure (p=0.001) and mean blood pressure (p=0.001) from pre-induction values than those who did not. CONCLUSION: Pre-treatment with a combination of fentanyl and midazolam is more effective than that with fentanyl or midazolam alone in reducing the incidence and severity of EIM. Myoclonus is associated with a higher post-induction haemodynamic variation.

Clinical Outcome and Prognosis of Young Patients with Mycosis Fungoides.

BACKGROUND/OBJECTIVES: Mycosis fungoides (MF) in young patients is rare and may have atypical presentations. There are limited data in these patients. The objective was to determine the clinical outcome and prognosis of young patients with MF. METHODS: A search of our institutional cancer registry database was conducted for patients diagnosed with MF at younger than 30 years of age. RESULTS: Our study included 74 patients (median age at diagnosis 25.5 yrs). Sixty-five (88%) presented with early stage disease and variants of MF (n = 44 [59%]), leading to a median delay in diagnosis of 2.5 years. Hypopigmented MF (n = 27 [36.5%]) was the most common variant, affecting predominantly African American (44.4% vs 19%; p = 0.02) and younger (20 vs 26 yrs; p < 0.001) patients. All patients with hypopigmented MF presented with early stage disease and were less likely to develop progressive disease (PD) than those with other variants (11% vs 34%; p = 0.03). Nineteen patients (26%) developed PD during a median follow-up of 3.5 years, which was associated with advanced-stage disease (89% vs 17%; p < 0.001), older age (>20 yrs) (31% vs 13%; p = 0.08), African American race (52.6% vs 20%; p = 0.009), and poikilodermatous presentation (p < 0.01). Overall survival was good (97.2% at 5 yrs, 95.9% at 10 yrs) despite the delay in diagnosis and atypical presentation. CONCLUSIONS: Progressive disease is associated with older age, African American race, the poikilodermatous variant, and advanced-stage disease. The hypopigmented variant is a common presentation in young patients and has an indolent disease course. Our study confirms an overall favorable prognosis in young patients with MF.

Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas.

Primary cutaneous CD8-positive aggressive epidermotropic T-cell lymphoma is a rare and poorly characterized variant of cutaneous lymphoma still considered a provisional entity in the latest 2016 World Health Organization Classification of Cutaneous lymphomas. We sought to better characterize and provide diagnostic and therapeutic guidance of this rare cutaneous lymphoma. Thirty-four patients with a median age of 77 years (range 19-89 years) presented primarily with extensive annular necrotic plaques or tumor lesions with frequent mucous membrane involvement. The 5-year survival was 32% with a median survival of 12 months. A subset of 17 patients had a prodrome of chronic patches prior to the development of aggressive ulcerative lesions. We identified cases with lack of CD8 or αß T-cell receptor expression yet with similar clinical and pathological presentation. Allogeneic stem cell transplantation provided partial or complete remissions in 5/6 patients. We recommend the term primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma as this more broad designation better describes this clinical-pathologic presentation, which allows the inclusion of cases with CD8 negative and/or αß/γδ T-cell receptor chain double-positive or double-negative expression. We have identified early skin signs of chronic patch/plaque lesions that are often misdiagnosed as eczema, psoriasis, or mycosis fungoides. Our experience confirms the poor prognosis of this entity and highlights the inefficacy of our standard therapies with the exception of allogeneic stem cell transplantation in selected cases.

Systemic therapy for cutaneous T-cell lymphoma: who, when, what, and why?

INTRODUCTION: CTCL are rare neoplasms. Optimal care requires integrated use of diagnostic and treatment modalities spanning multiple specialties. Current instruments for patient risk stratification and disease measurement across all anatomical compartments are suboptimal. A common treatment dichotomy between early (Dermatology) and advanced stage (Hematology-Oncology) has hindered accrual of long term outcome data. Thus, important facts about natural history, such as frequency and determinants of stage progression, and the impact of specific treatment modalities on these endpoints, are not known. Areas covered: One of the most important decisions in the management of CTCL is when to start systemic therapy and what agents to use. This review provides background information to understand why systemic therapy is needed and what goals are currently achievable. Expert commentary: Risk-adapted approaches, based on better knowledge of host and tumor biology, and more accurate disease measurement tools are needed to optimize the use of specific systemic therapies.

Anesthetic Management of Clinically Silent Familial Pheochromocytoma with MEN 2A: A Report of Four Cases.

Familial pheochromocytomas are commonly associated with multiple endocrine neoplasia type 2 (MEN 2) syndrome. Majority of the patients present with normal clinical and biochemical parameters in the preoperative period, the incidence of hypertension being only 50 %. Even though patients may be clinically asymptomatic, surveillance and proper preoperative evaluation is important, as surgery for associated tumors may precipitate a hypertensive crisis and result in severe complications. A family of 19 members, of which 12 were positive for MEN 2A syndrome, presented to our hospital. Seven of the 12 patients had pheochromocytoma and medullary thyroid carcinoma (MTC), while the other 5 had only raised plasma calcitonin levels. Two of the 7 patients presented with bilateral pheochromocytoma and underwent an open adrenalectomy. The other 5 patients had a left-sided adrenal tumor and underwent left laparoscopic adrenalectomy under combined general and epidural anesthesia. We present our experience with four of these five cases. We here state that how paucity of literature on perioperative preparation of clinically and biochemically silent pheochromocytomas led to serious intraoperative complications in one of four cases.

Long-term follow-up and management of small and medium-sized CD4+ T cell lymphoma and CD8+ lymphoid proliferations of acral sites: a multicenter experience.

BACKGROUND: Primary cutaneous CD4+ small-medium pleomorphic T cell lymphoma (SMPTCL) is a low-grade cutaneous T cell lymphoma. Its clinical and histopathologic features are comparable with those of CD8+ lymphoid proliferations (LPs) of the ear and acral sites. OBJECTIVES: We performed a retrospective analysis of patients with CD4+ SMPTCL or CD8+ LP to elucidate the clinical course, prognosis, and outcomes. METHODS: Demographic, clinical, and treatment data were reviewed. Histopathologic data based on architectural, cytomorphologic, and immunohistochemical features were assessed. Immunohistochemical staining for T and B cell markers was evaluated. RESULTS: A total of 25 patients including 22 with CD4+ SMPTCL and three with CD8+ LP were identified. All patients presented with a single lesion, predominantly on the head, neck, or upper trunk (84%). No patients showed extracutaneous disease at any evaluation. The most common histopathologic changes showed a dense nodular infiltrate of small cells with hyperchromatic nuclei without significant follicular or adnexal involvement. Patients were treated with excision (48%), local radiation (28%), or topical or intralesional steroids (24%). All patients achieved complete resolution of disease. Five patients demonstrated cutaneous relapse at new sites. CONCLUSIONS: The CD4+ SMPTCL/CD8+ LP subgroup usually presents with solitary lesions and demonstrates an indolent clinical course. Typical presentation, classic histopathology, widespread expression of follicular T helper cell markers, and loss of a T cell antigen are diagnostic features of CD4+ SMPTCL, whereas monomorphous CD8+ infiltrate without follicular T helper cell markers is consistent with CD8+ LP. Local skin-directed therapy is appropriate in these patients.

Managing Patients with Cutaneous B-Cell and T-Cell Lymphomas Other Than Mycosis Fungoides.

Cutaneous lymphomas (CL) are a heterogeneous group of neoplasms characterized with clinical and histopathological variation, as well as overlap with benign dermatoses. Diagnosis and treatment of CLs is challenging and often requires a multidisciplinary approach. However, prognostic knowledge of these conditions and awareness of treatment options can help optimize appropriate use of available regimens, thereby improving care for patients. Here, we review the most recent literature and outline treatment themes for managing patients with cutaneous B-cell and T-cell lymphomas other than mycosis fungoides.

Unusual variants of mycosis fungoides.

Conventional presentations of mycosis fungoides may be diagnostically challenging, particularly in light of the controversial boundaries defining the disease. Variant presentations of this cutaneous T-cell lymphoma add a further layer of complexity, requiring a sophisticated and informed perspective when evaluating lymphoid infiltrates in the skin. Herein we discuss well-defined (WHO-EORTC) variants pagetoid reticulosis, granulomatous slack skin and folliculotropic mycosis fungoides as well as less well-defined morphologic/architectural variants, and divergent immunohistochemical presentations of this typically indolent T-cell lymphoproliferative disease.

Hematopoietic Stem Cell Transplant for Mycosis Fungoides and Sézary Syndrome.

Mycosis fungoides (MF) and Sézary syndrome (SS) are common types of primary cutaneous T-cell lymphoma. Early-stage MF has a favorable prognosis and responds well to skin-directed regimens. Patients with advanced-stage MF, transformed MF, and SS are treated with combined systemic and skin-directed therapies. However, the disease is incurable with standard regimens, and frequent relapses are common. Owing to the lack of improvement in overall survival with standard regimens, hematopoietic stem cell transplant (HSCT) has been explored as a potential curative option. This article reviews the role of HSCT in MF/SS and discusses data regarding conditioning regimens, treatment-related complications, and outcomes.

Unilateral pulmonary edema during laparoscopic resection of adrenal tumor.

Despite technological, therapeutic and diagnostic advancements, surgical intervention in pheochromocytoma may result in a life-threatening situation. We report a patient who developed unilateral pulmonary edema during laparoscopic resection of adrenal tumor.