A case report of aggressive course of CD30+ primary cutaneous anaplastic large cell lymphoma.

INTRODUCTION: CD30+ primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is a rare T-cell neoplasm, and has been reported to present with an indolent behavior. The PC-ALCL with aggressive behavior has not been reported in the literature. PATIENT CONCERNS: We treated a patient with PC-ALCL that exhibited indolent behavior in the past 2 years and aggressive behavior within the last 3 months before presentation. DIAGNOSIS: Aggressive CD30+ primary cutaneous anaplastic large cell lymphoma. INTERVENTIONS: The radiotherapy regimen was individualized in terms of the target volume delineation and dose prescription, and the dose-response relationship was evaluated. OUTCOMES: The mean distance of microscopic infiltration was 14.1 mm in depth and 14.3 mm circumferentially. The lesion completely regressed after the delivery of 40 Gy in 20 fractions over 4 weeks. The tumor did not recur over the next year. CONCLUSION: An aggressive disease course is rare for indolent CD30+ PC-ALCL, which has similar histopathological characteristics as indolent PC-ALCL. The radiotherapy strategy should be individualized with curative intent.

Pediatric primary cutaneous anaplastic large cell lymphoma treated with brachytherapy.

Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is a CD30+ lymphoproliferative disorder that rarely occurs in children. Although there are currently no consensus guidelines for the treatment of cutaneous lymphoma in the pediatric population, the isolated form of PC-ALCL is typically managed by surgical excision or external beam radiation therapy. We report the case of a 6-year-old girl with primary cutaneous anaplastic large cell lymphoma that was treated with brachytherapy with no recurrence after 21 months of follow-up, suggesting that brachytherapy may be considered as a treatment for pediatric cutaneous large cell anaplastic lymphoma.

Radiotherapy of primary cutaneous anaplastic large cell lymphoma: our experience in 30 cases.

BACKGROUND: Since the observation of new cases of primary cutaneous anaplastic large cell lymphoma (PCALCL) with a seemingly aggressive clinical presentation and a favorable response to radiation therapy (RT), a review of our series has been performed to evaluate the results of RT. MATERIALS AND METHODS: The study is a retrospective analysis of 30 patients with PCALCL treated with conventional energy RT. RESULTS: About 55 fields of irradiation were performed. Complete clinical response (CCR) was obtained in 29 cases and a partial clinical response (PCR) in one. Two lesions had a marginal relapse at 7 and 37 months, respectively. In eight cases, new lesions appeared in the same body district and in six cases, in body sites at distance from the field of treatment. Total radiation dose ranged from 15 to 35 Gy (median 25 Gy). Follow-up range was 2-218 months (median 38.5 months) with a local control rate of 85% and a relapse-free rate of 49% at 5 years from RT. Three cases developed a nodal involvement. CONCLUSIONS: PCALCL belongs to the group of primary cutaneous CD30+ lymphoproliferative disorders, accounting for 25-30% of cutaneous T-cell lymphomas. RT is considered of choice as alternative to surgical excision of solitary lesions. Recent studies have focused on ideal dose to obtain local control indicating 30 Gy as adequate, but others have hypothesized that lower doses may suffice. Our study confirms the excellent role of RT in the local control of the disease with total doses ≤25 Gy.

Diffuse Primary Cutaneous Anaplastic Large Cell Lymphoma Treated by Rotational Total Skin Electron Beam Radiotherapy with Custom Shielding: Case Report.

INTRODUCTION: Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare T-cell malignancy typically presenting as a solitary lesion treated with radiotherapy. Diffuse PCALCL is rare, and treatment paradigms of diffuse PCALCL are poorly defined. CASE AND OUTCOMES: In this report, a 69-year-old male presented with progressive extensive truncal PCALCL resistant to brentuximab. The truncal lesions were treated with 36 Gy in 18 fractions by a novel approach using rotational electron beam radiation therapy with custom-made shielding. The treatment was well tolerated with expected dermatologic side effects managed supportively. All lesions achieved an initial complete response, and two sites within the treatment field recurred five months after treatment. DISCUSSION: This case adds to the limited literature on diffuse PCALCL and demonstrates an uncommon treatment approach to multifocal PCALCL using rotational electron beam radiation therapy with personalized shielding techniques. The treatment approach here was well tolerated by the patient with initial complete response at all sites. Maximal sparing was especially critical in this patient because of a history of previous head and neck irradiation. Shielded areas were validated by optically stimulated luminescent dosimeters showing dose reduction, confirming the utility of this method. CONCLUSION: Rotational total skin electron beam with personalized shielding may be generalizable to cutaneous malignancies including PCALCL presenting with diffuse skin involvement. Further investigation of diffuse PCALCL is merited to optimize treatment strategies.

ALK-positive primary cutaneous anaplastic large cell lymphoma: a case report and review of the literature.

Anaplastic large cell lymphoma (ALCL) limited to the skin is a distinct disease that is designated primary cutaneous ALCL (pcALCL). It has an indolent course with a significantly better prognosis compared to systemic ALCL (sALCL). Anaplastic lymphoma kinase (ALK) expression in lesions of cutaneous ALCL is classically considered to be a marker for skin involvement by sALCL. However, recent reports of patients with ALK-positive pcALCL challenge this concept and raise prognostic and therapeutic dilemmas. Herein, we report a case of ALK-positive pcALCL in a 45-year-old woman who was treated with local radiotherapy. We review previously reported cases in the literature to better characterize this rare variant. Overall, the rates of cutaneous recurrence, systemic dissemination, and disease-related mortality in ALK-positive pcALCL do not differ from those previously reported in pcALCL. ALK-positive pcALCL is diagnosed at younger age and has a better disease course in children compared to adults with lower incidences of skin recurrence and progression to systemic disease. We conclude that ALK-positivity in cutaneous ALCL does not necessarily imply systemic disease. ALK-positive pcALCL has an excellent prognosis and should be treated by excision and/or radiotherapy. However, patients must remain under close long-term follow-up as recurrence and progression to systemic disease may occur.

Recommendations for the Optimal Radiation Dose in Patients With Primary Cutaneous Anaplastic Large Cell Lymphoma: A Report of the Dutch Cutaneous Lymphoma Group.

PURPOSE: To determine the optimal radiation dose for treatment of primary cutaneous anaplastic large cell lymphoma (C-ALCL) with solitary or localized, multifocal or recurrent skin lesions. METHODS AND MATERIALS: In this multicenter study, patients with C-ALCL who had been treated with radiation therapy (RT) between 1984 and 2016 were retrieved from the Dutch registry of cutaneous lymphomas. Distinction was made between patients first presenting with solitary or localized lesions (n=63), with multifocal skin lesions (n=6), and patients with a skin relapse (n=22). Radiation doses, treatment response, and follow-up were evaluated. Radiation doses were categorized as low-dose (≤20 Gy), intermediate-dose (21-39 Gy), and high-dose (≥40 Gy) RT. RESULTS: Of 63 patients presenting with solitary or localized skin lesions, 61 (97%) showed a complete response (CR). There were no differences in CR between low-dose (16 of 17), intermediate-dose (15 of 15), and high-dose RT (30 of 31). After a median follow-up of 46 months, 30 of 63 patients (48%) had a relapse, but in-field relapses were never observed. Six of 6 patients (100%) initially presenting with multifocal skin lesions showed a CR (3 of 3 low-dose, 2 of 2 intermediate-dose, 1 of 1 high-dose RT). After a median follow-up of 27 months, 3 of 6 patients had a relapse. Treatment of 33 skin relapses in 22 patients showed no differences in CR between low-dose (18 of 19), intermediate-dose (6 of 6), and high-dose RT (8 of 8). In the last 10 years there has been a decrease in radiation dose used in the treatment of C-ALCL. Treatment of multifocal and recurrent lesions with a dose of 8 Gy (2 × 4 Gy) resulted in CR of 17 of 18 lesions. CONCLUSIONS: Our results show that a radiation dose of 20 Gy (8 × 2.5 Gy) is effective in patients presenting with solitary or localized skin lesions. For patients with multifocal skin lesions and patients with a skin relapse, a dose of 8 Gy (2 × 4 Gy) may be sufficient.

Radiation Therapy for Cutaneous T-Cell Lymphomas.

Radiation therapy is an extraordinarily effective skin-directed therapy for cutaneous T-cell lymphomas. Lymphocytes are extremely sensitive to radiation and a complete response is generally achieved even with low doses. Radiation therapy has several important roles in the management of mycosis fungoides. For the rare patient with unilesional disease, radiation therapy alone is potentially curative. For patients with more advanced cutaneous disease, radiation therapy to local lesions or to the entire skin can effectively palliate symptomatic disease and provide local disease control. Compared with other skin-directed therapies, radiation therapy is particularly advantageous because it can effectively penetrate and treat thicker plaques and tumors.

Primary cutaneous CD30-positive anaplastic large-cell lymphoma of the external auditory canal.

Primary cutaneous T-cell lymphoma is rare. Cutaneous lymphoma is defined as primary when there is an absence of nodal or systemic disease during the first 6 months following diagnosis. We report what we believe to be the first documented case of a primary cutaneous CD30-positive anaplastic large-cell lymphoma of the external auditory canal. The patient was an elderly woman who presented with progressively worsening right otalgia and hypoacusis. Otoscopy revealed an erythematic, ulcerative, nonbleeding, localized lesion in the anterosuperior area of the external auditory canal. The patient underwent an excisional biopsy, and after the diagnosis was established, she underwent 22 sessions of radiotherapy. During follow-up, she exhibited no evidence of recurrence.

Anaplastic large cell lymphoma of scrotal skin.

Cutaneous T cell lymphoma (CTCL) is an uncommon diverse group of lympho-proliferative disorders involving the skin. They vary considerably in clinical presentation, microscopic features and immunophenotyping. The diagnosis is challenging, zealous, and often not easy. CD30+ve anaplastic large cell lymphoma is extremely rare. Its clinical spectrum varies from a solitary unifocal skin lesion of excellent prognosis to a multi focal systemic disease having a poor out come. The diagnosis is quite cumbersome, and often difficult. The differential diagnosis include from benign skin lesions to secondary cutaneous involvement by lymphoma. A correct diagnosis is integral with a complete metastatic/staging work up to avoid over treatment. The treatment options depend on extent of disease involvement and include surgical excision, surveillance, local radiotherapy, and systemic chemotherapy. The prognosis is good with unifocal local disease. We present here a very rare case of CD30+ ALCL of scrotal skin, in a middle aged male patient.

Primary cutaneous anaplastic large-cell lymphoma.

Primary cutaneous anaplastic large-cell lymphoma (ALCL) is a form of cutaneous T-cell lymphoma that is characterized by solitary or localized nodules or plaques. Histopathologic features include a diffuse, non-epidermotropic infiltrate with cohesive sheets of large anaplastic CD30+ tumor cells. This entity must be distinguished from systemic ALCL with cutaneous involvement and lymphomatoid papulosis. Treatment modalities include clinical monitoring, radiation therapy, and surgical excision, with systemic chemotherapy reserved for disseminated or extracutaneous disease.

Neutrophil/eosinophil-rich type of primary cutaneous anaplastic large cell lymphoma: a clinicopathological, immunophenotypic and molecular study of nine cases.

AIMS: To elucidate the clinicopathological, immunophenotypic and molecular features of neutrophil/eosinophil-rich primary cutaneous anaplastic large cell lymphoma (CALCL), and to emphasize the cutaneous manifestations, differential diagnosis and prognosis of this peculiar entity. METHODS AND RESULTS: We described the clinical presentations, histopathology, immunophenotype, molecular features and follow-up courses of nine neutrophil/eosinophil-rich CALCL cases. Various clinical lesions including multiple nodules, plaques and solitary exophytic masses with or without ulceration or crusting were noted in nine patients. Two patients died of disease progression, with one developing multiple lymph node involvement. Histologically, cohesive sheets or small clusters of neoplastic cells were admixed with large numbers of neutrophils and/or eosinophils, representing 10-40% of cells per high-power field. All nine cases showed T-cell phenotypes. The frequency of rearranged TCRB, TCRG and TCRD genes in six cases with available paraffin-embedded tissue was 100%, 83% and 33%, respectively. CONCLUSIONS: Neutrophil/eosinophil-rich CALCL should be differentiated from various infectious and non-infectious diseases, especially from non-neoplastic cutaneous CD30+ infiltrates rich in neutrophils and eosinophils. Precise correlation of clinical presentation, morphological features, phenotypic and molecular analysis can help to establish the correct diagnosis. Whether this rare variant has a significantly different prognosis from classical CALCL needs further investigation.

Treatment of primary cutaneous CD30+ anaplastic large-cell lymphoma with radiation therapy.

PURPOSE: Primary cutaneous CD30+ anaplastic large-cell lymphoma (CALCL) is a relatively rare and indolent variant of cutaneous T-cell lymphoma (CTCL). This report examines the response of localized disease to radiation alone. METHODS: The Yale Cancer Center records were examined, and all patients with CTCL from January 1, 2001, to September 1, 2006, evaluated in the Department of Therapeutic Radiology were identified. Only those patients with localized or single CALCL lesions, no clinical evidence or history of lymphomatoid papulosis, no history of other CTCLs, no history of other skin disorders, lack of lymph node involvement, unambiguous pathology reports, and treatment with radiation alone were included. RESULTS: Eight patients were identified. Median age was 67 years, and gender was split evenly. Patients received radiation ranging from 34 to 44 Gy in 2-Gy fractions. Most patients (5 of 8) received 40 Gy, using 6 to 9 MeV electrons with 0.5 to 2 cm of bolus. All patients had a complete response. All patients were without evidence of disease at the most recent follow-up (median follow-up, 12 months). Radiation therapy was well tolerated, and the only recorded toxicity was Grade I to II dermatitis. CONCLUSIONS: Radiation therapy alone for localized CALCL is very well tolerated and clinical response is excellent. A dose of 40 Gy in 2-Gy fractions seems to be well tolerated and effective in inducing a complete response. Lower doses may be effective in achieving the same result, but data are not available. Longer follow-up is necessary before conclusions regarding durable disease-free survival can be made.