Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is rare among skin malignancies. C-ALCL usually manifests as reddish or violet nodules. Surgical excision or radiation therapy is generally considered as first-line therapy, but a clinically aggressive disease may require multiagent chemotherapy. Establishing a proper diagnosis of C-ALCL is challenging but should be made to avoid inappropriate treatment and its consequences. The authors report a case of medically resolved C-ALCL in an 81-year-old man presented with well-defined nodular lesions on the forehead.
Aged, 80 and over ; Diagnosis ; Drug Therapy ; Forehead ; Humans ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell ; Skin ; Viola

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare primary cutaneous lymphoma that is predominantly composed of large lymphoid cells that express the CD30 antigen. The skin lesion of PCALCL is usually single, ulcerative, and located on the trunk or extremities and rarely the palm. A 25-year-old woman presented with a plaque on the left palm for 20 days. The plaque was walnut-sized and purple to gray colored with erosion in the center. Histopathologic examination showed infiltration of large atypical cells in the dermis. The large tumor cells showed positivity for CD3, CD4, and CD30 and negativity for CD8, CD20, epithelial membrane antigen, and anaplastic lymphoma kinase. PET-CT showed no other hypermetabolic lesion except that on the left palm, and we finally arrived at a diagnosis of PCALCL. The patient was treated with an intralesional injection of methotrexate (25 mg/mL, 0.45 cc). After 3 months of treatment, the walnut-sized plaque had disappeared and a peripheral hyperpigmented patch remained.
Adult ; Antigens, CD30 ; Dermis ; Diagnosis ; Extremities ; Female ; Humans ; Injections, Intralesional ; Lymphocytes ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell* ; Methotrexate ; Mucin-1 ; Phosphotransferases ; Skin ; Ulcer

Humans ; Lymphoma, Large-Cell, Anaplastic ; diagnosis ; enzymology ; therapy ; Receptor Protein-Tyrosine Kinases

OBJECTIVETo analyze the clinical features and prognostic factors of children's anaplastic large cell lymphoma (ALCL), summarize the therapeutic effect and toxicities.

METHODA total of 38 ALCL patients admitted to Beijing Children's Hospital from Jan. 2003 to Apr. 2010 were treated with BCH-ALCL-2003 regimen (modified from HK-ALCL-2000).

RESULTThirty-four cases were ALK(+), male:female ratio = 2.16:1. The median age was 9 years; 86.8% had B symptoms. 94.7% evolved to Stage III and IV on admission. The median follow-up duration was 48 months (12 to 99 months). Median event-free survival (EFS) time was 43 months. Thirty-four patients (89.5%) achieved a remission. The disease relapsed in 3 patients within 20 months after diagnosis. Estimated 4-year EFS was (81.2 ± 6.4)%, estimated 4-year overall survival (OS) rate was (86.4 ± 5.7)%. Univariate analysis indicated that the unfavorable prognostic factors included: more than 3 extra nodal involvement, hepatosplenomegaly (> 3 cm), elevated lactate dehydrogenase (LDH), stage IV, hemophagocytosis in bone marrow, and age < 3 years. The major toxicity was myelosuppression and mucositis. no chemotherapy related death occurred.

CONCLUSION(1) Childhood ALCL patients often have B symptoms and extranodal involvement. (2) In the study, therapeutic effects was good. The disease relapsed mostly within the first 2 years, maintenance therapy with vinblastine is necessary. (3) The regimen is safe to patients.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Lymphoma, Large-Cell, Anaplastic ; diagnosis ; therapy ; Male ; Prognosis ; Treatment Outcome

To study the differentiating cytomorphological features of Hodgkin lymphoma (HL) and anaplastic large cell lymphoma (ALCL) using fine needle aspiration cytology (FNAC), cytomorphological features of 16 patients with HL (n=8) or ALCL (n=8) were analyzed. In the initial cytological diagnosis prior to biopsy, HLs were properly diagnosed in 4 out of 8 cases (4 HL, 2 atypical, 2 benign), whereas all ALCL were diagnosed as malignancies. However, correct diagnosis of non-Hodgkin lymphoma (NHL) was made in only two ALCL patients (2 NHL, 1 HL, 1 sarcoma, 4 malignancy without specific type). Overall, the percentage of large abnormal cells ranged from 30% to 90% in ALCL except for one case, whereas it was less than 5% in all 8 HL. A spectrum of atypical cells was more characteristic of ALCL. In contrast, HL showed an sharp difference between reactive lymphoid cells and neoplastic ones (bimorphic pattern). Moreover, the emergence of kidney-shaped abnormal cells or wreath-like multinucleated cells was helpful in diagnosing ALCL. The combination of thesefeatures would be useful in differentiating HL and ALCL. Nevertheless, these two types of lymphomas cannot be definitely distinguished based on cytomorphological features alone. Therefore, the aim of FNAC would be to suggest a specific diagnosis and indicate the need for a biopsy.
Biopsy ; Biopsy, Fine-Needle* ; Diagnosis ; Hodgkin Disease* ; Humans ; Lymphocytes ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic* ; Lymphoma, Non-Hodgkin ; Sarcoma

Asthma ; diagnosis ; Bronchial Neoplasms ; diagnosis ; Child ; Diagnosis, Differential ; Female ; Humans ; Lymphoma, Large-Cell, Anaplastic ; diagnosis ; Tracheal Neoplasms ; diagnosis

PURPOSE: Primary cutaneous anaplastic large cell lymphoma is rarely encountered in the lower eyelids. We report a patient with primary cutaneous anaplastic large cell lymphoma arising from the lower eyelid. METHODS: A 39-year-old man presented with a relatively fast growing mass on the center of his left lower eyelid for one month. The mass did not respond to local injection of triamcinolone at a local clinic. The lesion appeared as a solitary reddish nodule with ulceration, was non-tender, round, crusted, and measured 13 mm x 11 mm x 5 mm. Well- developed superficial vessels were found on the surface of the nodule. An incisional biopsy was performed. RESULTS: Histologic examination revealed that the bulk of the infiltrate was in the papillary and reticular dermis. Tumor cells had abundant, well-defined cytoplasm and pleomorphic nuclei with multiple nucleoli. The majority of the neoplastic cells showed immunoreactivity for CD 30 (Ki-1) along the cell membrane. A histopathological diagnosis of primary cutaneous anaplastic large cell lymphoma was made. CONCLUSIONS: Most cases of primary cutaneous anaplastic large cell lymphoma arise from the body and extremities. However, since primary cutaneous anaplastic large cell lymphoma may occur in the eyelid, it should be differentiated from nodular and relatively fast growing inflammatory tumors despite local steroid treatment.
Adult ; Biopsy ; Cell Membrane ; Cytoplasm ; Dermis ; Diagnosis ; Extremities ; Eyelids ; Humans ; Lymphoma, Primary Cutaneous Anaplastic Large Cell* ; Triamcinolone ; Ulcer

PURPOSE: Anaplastic large cell lymphoma, has the following three characteristics of a malignant lymphoma; 1) An irregular large nucleus, called pathologic atypical cells, 2) Eosinophilic cytoplasm, 3) Immunologically positive for Ki-1. Anaplastic large cell lymphoma occurs mostly in the lymph nodes, but about 40% has been observed to occur in other tissues. Skin is the one of the main sources of origin and it is called 'primary cutaneous anaplastic large cell lymphoma'. METHODS: A 69-year-old male patient with an erythematous nodule, sized 1.5 X 1.7 cm on his right hand dorsum was excised under local anesthesia and on biopsy was diagnosed as 'Dermatofibrosarcoma Protuberans'. Three months after the local excision and biopsy, same natured mass reoccurred in the same region, and then spontaneous regressed after three weeks. However, metastatic large mass of 4.0 X 5.0 cm, of same nature was observed on the elbow. The large mass was operated with wide excision and biopsy. RESULTS: On final diagnosis, with an immunofluorescent stain with CD30(Ki-1), 'Primary cutaneous large cell lymphoma' was made. After follow up for three years, we did not observed recurrence and metastasis. CONCLUSION: We have reported that we have diagnosed primary cutaneous large cell lymphoma and treated without recurrence and metastasis.
Aged ; Anesthesia, Local ; Biopsy ; Cytoplasm ; Diagnosis ; Elbow ; Eosinophils ; Follow-Up Studies ; Hand ; Humans ; Lymph Nodes ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell* ; Male ; Neoplasm Metastasis ; Recurrence ; Skin

The diagnosis of primary cutaneous lymphoma is based on a combination of clinical, histological, immunophenotypic and genetic criteria. Nineteen cases of primary cutaneous lymphomas were studied for clinicopathologic, immunophenotypic, and genetic features. Seventeen (89%) cases were T cell origin and two cases (11%) were B cell origin. CD30-positive cutaneous lymphoproliferative disorder was the most frequent subtype, occupying 42% (8 cases) of the cases. CD8 was positive in 5 cases consisting of 3 cutaneous T cell lymphomas and 2 anaplastic large cell lymphomas. CD4 was positive in 2 cases of mycosis fungoides and 3 cases of lymphomatoid papulosis. Six (67%) of 9 cases of cutaneous T cell lymphoma were positive for TIA-1. Ten (83%) out of 12 cases showed clonal rearrangements of TCR gamma genes, however, one T/NK cell lymphoma and one anaplastic large cell lymphoma did not. EBV association was detected only in T/NK cell lymphomas among 10 cases examined. In conclusion, our study showed higher proportion of CD30-positive lymphoproliferative disorders and less frequent mycosis fungoides in Korea compared to the incidences in Western countries. Our immunostaining results suggested that mycosis fungoides and lymphomatoid papulosis are CD4-positive T cell origin, however, the remaining primary cutaneous T cell lymphoma is predominantly CD8-positive cytotoxic T cell origin.
Diagnosis ; Genes, T-Cell Receptor gamma ; Herpesvirus 4, Human ; Incidence ; Korea ; Lymphoma* ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Mycosis Fungoides

A 29-year-old Korean man presented with erythematous nodules on his right arm and left leg. He had been diagnosed with mycosis fungoides 10 years prior and was treated with phototherapy continuously. A diagnostic skin biopsy was performed, and the histopathologic findings of the specimen revealed dense infiltration of atypical large lymphoid cells through the entire dermis. These cells showed positive CD3 and CD30 staining. Despite the atypical finding that T cells represented less than 75% of the infiltrated cells, primary cutaneous CD30 positive anaplastic large cell lymphoma was ultimately diagnosed based on the overall consideration of the clinical features and favorable prognosis. Because primary cutaneous CD30 positive anaplastic large cell lymphoma has a favorable prognosis, it should be differentiated from large cell transformation of mycosis fungoides, which has a poor prognosis and requires aggressive treatment. However, this differential diagnosis is challenging. Herein, we report a rare case of primary cutaneous CD30 positive anaplastic large cell lymphoma with mycosis fungoides differentially diagnosed from large cell transformation.
Adult ; Arm ; Biopsy ; Dermis ; Diagnosis, Differential ; Humans ; Leg ; Lymphocytes ; Lymphoma, Large-Cell, Anaplastic* ; Mycosis Fungoides* ; Phototherapy ; Prognosis ; Skin ; T-Lymphocytes