A case of primary cutaneous anaplastic large cell lymphoma on eyelid.

Primary cutaneous anaplastic large cell lymphoma is a kind of cluster of differentiation 30+ primary cutaneous lymphoproliferative disorders with a relatively good prognosis in the absence of high-stage disease. Primary cutaneous anaplastic large cell lymphoma shows a higher frequency in males and commonly affects the head and neck. Palpebral involvement is very rare. We present a 42-year-old lady patient with primary cutaneous anaplastic large cell lymphoma involving the eyelid which was initially misdiagnosed as stye. The patient underwent a total excision of the lesion and showed complete regression of the lesion after surgery without any other treatment. There was no evidence of local or systemic disease during follow-up after nine months.

ALK-Negative Primary Cutaneous Anaplastic Large Cell Lymphoma With Systemic Involvement or Systemic ALCL With Cutaneous Lesion. A Diagnostic Dilemma.

ABSTRACT: Primary cutaneous anaplastic large-cell lymphoma (C-ALCL) is a cutaneous CD30-positive lymphoproliferative disorder. The patients usually present with single or multiple cutaneous nodules or papules and about 10% cases present with extracutaneous manifestations, which are predominantly in the form of regional lymph nodal involvement. Visceral involvement especially pulmonary or hepatic involvement in C-ALCL is only rarely described in the scientific literature. Approximately 20%-42% cases show spontaneous regression, about 50% cases may recur; however, C-ALCL generally carries a good prognosis. We present a rare case of primary C-ALCL in a 66-year-old man with regional lymph nodal and hepatic involvement. Differential diagnostic entities are discussed in this report with the review of the literature.

Diagnosis and Management of Cutaneous Lymphomas Including Cutaneous T-cell Lymphoma.

The cutaneous lymphomas are malignancies of T-cell and B-cell lymphocytes in which the skin is the primary organ of involvement. The cutaneous T-cell lymphomas include variants that can mimic the presentation of common skin diseases or arthropod bites. Mycosis fungoides, the most common cutaneous T-cell lymphoma, usually presents as fixed asymptomatic patches or plaques in sun-protected areas. The cutaneous B-cell lymphomas have fewer variants that often present as papules or nodules that can mimic nonmelanoma skin cancers. Some therapies for cutaneous lymphoma have unique side effects such as central hypothyroidism, hyperlipidemia, and peripheral neuropathy.

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.

Linfoma primario cutáneo de células T epidermotropo agresivo CD8+ / Primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma

El linfoma de células T epidermotropo agresivo CD8+ o linfoma de Berti es un subtipo infrecuente de los linfomas primarios cutáneos, descripto en 1999. Aún se considera una entidad provisional en la última clasificación de linfomas cutáneos primarios de la Organización Mundial de la Salud. Predomina en los hombres adultos y se manifiesta con pápulas, placas hiperqueratósicas y tumores ulcerados, diseminados y de inicio agudo. En la histopatología, es característica la presencia de un infiltrado de linfocitos atípicos medianos CD8 +, con epidermotropismo de patrón pagetoide. Tiene un comportamiento agresivo y es de mal pronóstico, con una sobrevida a los 5 años menor del 40%. Si bien esta entidad es un reto terapéutico, el tratamiento inicial se basa en poliquimioterapia y/o trasplante de células hematopoyéticas.

The aggressive epidermotropic CD8+ T-cell lymphoma or Berti's lymphoma, is a rare subtype of primary cutaneous lymphomas, first described in 1999. It is still considered a provisional entity by the latest World Health Organization classification of cutaneous lymphomas. Patients are commonly adults with a male predominance and it is characterized by widespread rapid evolving papules, hyperkeratotic annular plaques and ulcerated tumors. Histopathologicfindingsarecharacteristic,withaninfiltrate of medium-sized CD8+ atypical lymphocytes involving the epidermis with a pagetoid pattern. Ithasanaggressivebehaviorandtheprognosisispoor with a 5-yearsurvival less than 40%. Instead these disease represents a therapeutic challenge, the initial treatment consists on polychemotherapy and hematopoietic stem cell transplantation.

A case of lymphomatoid papulosis type E in a young adult: An uncommon entity.

Lymphomatoid papulosis (LyP) type E is a rare variant of the primary cutaneous CD30+ lymphoproliferative disorders, characterized clinically by large necrotic eschar-like lesions and histopathologically by angiodestructive and angioinvasive infiltrates of CD30+ lymphocytes. As in other forms of lymphomatoid papulosis, type E lesions may undergo spontaneous regression after weeks, with frequent recurrences. We report a 21-year old male with an angiodestructive infiltrate of CD30+ lymphocytes manifesting as a papular eruption rather than ulceration, and suggest that this clinical phenotype might be related to the presence of CD4+ lymphocytes in the inflammatory cell infiltrate.

Primary cutaneous anaplastic large-cell lymphoma of the eyelid: report of two cases and review of the literature.

PURPOSE: Two new cases of primary cutaneous CD30+ anaplastic large-cell lymphoma (cALCL) of the eyelid are reported; these are analysed alongside existing cases to identify challenges relating to the diagnosis and management of such rare lesions. MATERIAL AND METHODS: A review of existing literature on the PubMed database is conducted using the keywords: 'eyelid lymphoid proliferations', 'lymphoma of the eyelid', and 'primary cutaneous CD30+, ALK-anaplastic large-cell lymphoma of the eyelid'. Two new cases of cALCL are reported. Cases where patients present solely with a nodular periocular lesion are analysed for recurrence and survival rate. RESULTS: Two new patients with a painless ulcerated nodule on the upper eyelid receive a confirmed diagnosis of cALCL after undergoing an excisional biopsy. The first, elderly patient has spontaneous remission; the second patient, with a concomitant chronic infection of hepatitis C virus (HCV), presents a more diffuse disease at the onset and requires radiotherapy. Together with 13 patients a primary cALCL identified from 11 previous studies, this constitutes a cohort of 15 patients. Of these, 10 present with an exclusively nodular lesion of the eyelid and four experience disease recurrence; no deaths from cALCL are reported. CONCLUSION: Differential diagnosis between primary cALCL and lymphomatoid papulosis is essential and requires careful consideration of clinical and pathologic features. Radiologic staging examination is crucial in order to exclude systemic ALCL, particularly for patients with comorbidity. Though cALCL has the pathological features of a malignant lesion, the prognosis seems favourable for patients; a relatively high percentage even experience spontaneous resolution.

Lymphomatoid Papulosis and Other Lymphoma-Like Diseases.

Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica are the 2 main subtypes of pityriasis lichenoides. They represent the acute and chronic forms of the disease; both may have clonal T cells. Several treatment modalities are used, but it has been difficult to determine efficacy because of the possibility of spontaneous remission. Cutaneous CD30+ lymphoproliferative disorders constitute many cutaneous T-cell lymphomas and comprise lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma (ALCL). Both have an excellent prognosis. Lymphomatoid papulosis often only requires observation or treatment of symptoms. First-line therapies for primary cutaneous ALCL are surgical excision or radiotherapy.

Complete Resolution of Primary Cutaneous Anaplastic Large Cell Lymphoma With Topical Imiquimod

Primary cutaneous anaplastic large cell lymphoma (pc-ALCL) is a CD30+ subtype of cutaneous T-cell lymphoma. It typically has a very favorable prognosis; however, traditional treatment can be expensive, invasive, and associated with significant adverse events. Imiquimod is a topical toll-like receptor approved by the Food and Drug Administration (FDA) for genital warts, actinic keratosis, and primary superficial basal cell carcinoma. In previous case reports, imiquimod has been shown to be effective against pc-ALCL. We present a case of complete resolution of pc-ALCL within 8 weeks with topical imiquimod and review the current literature. J Drugs Dermatol. 2019;18(5):460-462.

Molluscum contagiosum infection with features of primary cutaneous anaplastic large cell lymphoma.

CD30+ T cell pseudolymphomas (CD30+ PSL) are a group of benign inflammatory cutaneous disorders that can develop in settings of viral infections or drug reactions. Owing to their histological similarities to malignant lymphomas, these benign infiltrates are occasionally misdiagnosed as malignant, causing significant concerns for patients and physicians. Herein, we report a patient with CD30+ PSL associated with molluscum contagiosum whose initial biopsy revealed atypical large CD30-expressing cells, leading to a misdiagnosis of primary cutaneous anaplastic large cell lymphoma and referral to our cutaneous lymphoma clinic. We report this case to demonstrate that reactive CD30+ infiltrate associated with molluscum contagiosum can be mistaken for T-cell lymphomas and patients should be reassured in these cases.

Primary cutaneous anaplastic large cell lymphoma arising from a long-standing erythema nodosum: A case report.

RATIONALE: Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a rare subtype of non-Hodgkin lymphoma, which is rarely associated with erythema nodosum (EN). PATIENT CONCERNS: A 57-year-old woman complained of recurrent rashes involving her abdomen, back, upper and lower limbs for over 20 years, with severity in symptoms for 2 weeks. DIAGNOSES: The first skin biopsy was performed in 2011 in another hospital and she was diagnosed idiopathic EN. The second skin biopsy was performed in 2014 and she was diagnosed as pcALCL with stage IA. INTERVENTIONS: She was treated with oral prednisone, cyclosporine, and thalidomide. OUTCOMES: One month later, the ulcerative lesion was decreased in size and became smooth and the patient achieved partial remission. She is still under treatment and has been monitored closely for 4 years. LESSONS: The case suggested that stimulation of inflammation in the skin lesions for a long period might be related to clonal transformation into pcALCL and hence should be closely monitored. Immunosuppressive treatment may be effective and safe for patients with pcALCL at early stages.

Primary cutaneous anaplastic large-cell lymphoma: A case report.

RATIONALE: Primary cutaneous anaplastic large-cell lymphoma (C-ALCL) is a rare cancer belonging to the group of primary T-cell lymphoproliferative diseases. C-ALCL is characterized by the presence of single or multiple ulcerated lesions on the skin's surface. PATIENT CONCERNS: This is the case of a 73-year-old man who reported to the Clinic of Cranio-Maxillofacial and Oral Surgery and Implantology, Medical University of Warsaw, owing to a skin tumor in the right parotideomasseteric region, initially diagnosed as discoid lupus erythematosus. During treatment for discoid lupus erythematosus, biopsy was repeated because of significant disease progression and dynamic tumor growth. Histopathological examination revealed the presence of pilomatrix carcinoma (trichilemmal carcinoma). Because of the discrepancy between clinical and histopathological findings, the tumor specimen was submitted to another facility, wherein lymphoma infiltration by anaplastic large cells was found in the dermis and subcutaneous tissue. DIAGNOSIS: C-ALCL. INTERVENTIONS: The patient was transferred to the Lymphoid Tumours Clinic of the Maria Sklodowska Curie Memorial Cancer Centre and Institute of Oncology in Warsaw, where chemotherapy was initiated. OUTCOMES: After 4 cycles of chemotherapy, a complete remission of skin lesions was achieved. During the 5-year follow-up, no recurrence occurred. LESSONS: C-ALCL is a rare type of cancer. Misdiagnosis can lead to inappropriate therapy and result in disease progression or unnecessary harm to the patient.

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.