Primary cutaneous anaplastic large cell lymphoma is a rare non-Hodgkin's lymphoma.The tumor cells have the characteristics of anaplastic cells,expressing CD30 but not anaplastic lymphoma kinase.In this study,we reported a case of primary cutaneous anaplastic large cell lymphoma in a Tibetan child and summarized the clinicopathological features,aiming to strengthen the understanding of this disease.
Child ; Humans ; Ki-1 Antigen ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Skin Neoplasms/pathology*

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare subtype of primary cutaneous lymphoma with a favorable prognosis. Primary cutaneous CD30+ lymphoproliferative disorders, which include C-ALCL and lymphomatoid papulosis, are the second most common group of cutaneous T-cell lymphomas. C-ALCL is comprised of large cells with anaplastic, pleomorphic, or immunoblastic cytomorphology, and indeed, more than 75% of the tumor cells express the CD30 antigen. C-ALCL clinically presents with solitary or localized reddish-brown nodules or tumors, and sometimes indurated papules, and they may be with ulceration covering with dark eschar. Multifocal lesions are seen in 20% of the patients. Extracutaneous dissemination, which mainly involves the regional lymph nodes, occurs in 10% of patients. A 69-year-old man noticed a mild elevated cutaneous lesion containing central ulceration covering with brownish black necrotic tissue on the right lower lip, and the lesion was surgically removed. After the first operation, another skin lesion was developed and the histological examination confirmed the diagnosis, C-ALCL. Eight specimens were excised during the 7-month follow-up period. The patient started the treatment with low-dose oral methotrexate (15 mg/wk) and there was no recurrence for 11 months.
Aged ; Antigens, CD30 ; Diagnosis ; Follow-Up Studies ; Humans ; Lip ; Lymph Nodes ; Lymphoma ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Methotrexate ; Prognosis ; Recurrence ; Skin ; Ulcer

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

CD30+ lymphoproliferative disorders (LPD) represent a spectrum of T-cell lymphoma including lymphomatoid papulosis and anaplastic large cell lymphoma (ALCL). Epidermis overlying cutaneous CD30+ LPD often shows epidermal hyperplasia, hyperkeratosis, crusting, and ulceration and it is difficult to distinguish from carcinoma such as keratoacanthoma (KA) or squamous cell carcinoma (SCC). Several cases of pseudocarcinomatous hyperplasia mimicking KA or SCC in CD30+ LPD have been reported. The relationship between CD30+ LPD and epithelial proliferations has not yet well understood. It was reported that a variety of mediators, including epidermal growth factor (EGF), transforming growth factor-α and EGFR from CD30+ LPD could attribute to epidermal hyperplasia. However, separate and distinct SCC occurring in CD30+ LPD has rarely been reported. Herein, we present a rare case of coexistence of SCC and cutaneous ALCL located on the same region.
Carcinoma, Squamous Cell* ; Epidermal Growth Factor ; Epidermis ; Epithelial Cells* ; Hyperplasia ; Keratoacanthoma ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell* ; Lymphoma, T-Cell ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Ulcer

No abstract available.
Histiocytoma, Benign Fibrous* ; Humans ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell*

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare cutaneous T-cell lymphoma. Most cases are composed of large anaplastic cells. However, it presents a wide spectrum of histologic patterns. In the small cell variant, a small-sized pleomorphic cell morphology can be seen. A 74-year-old woman presented with an 8-month history of asymptomatic ulcerative plaque and satellite nodule on the right calf. Her past medical history was not specific. The histologic findings on punch biopsy specimens showed a malignant small round cell tumor on both lesions. The tumor cells had large pleomorphic nuclei with multinucleation and some eosinophilic cytoplasm. We performed immunohistochemical staining to rule out neuroectodermal tumor, neuroendocrine tumor, melanoma, lymphoma, and so on. However, the staining results were negative for pancytokeratin, CD3, CD20, CD99, chromogranin A, synaptophysin, CD56, ALK, HMB45, desmin, kappa, lambda, myoglobin, and S-100 protein. CT, MRI, and PET-CT were negative for extracutaneous involvement. Total excision was done, and additional immunohistochemical staining was performed to confirm the origin of the tumor. Staining results for vimentin, LCA, CD4, and CD30 were positive. We concluded that these findings were consistent with the small cell variant CD30+ PCALCL, which occurs rarely.
Aged ; Biopsy ; Chromogranin A ; Cytoplasm ; Desmin ; Eosinophils ; Female ; Humans ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic* ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell, Cutaneous ; Magnetic Resonance Imaging ; Melanoma ; Myoglobin ; Neuroectodermal Tumors ; Neuroendocrine Tumors ; S100 Proteins ; Synaptophysin ; Ulcer ; Vimentin

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare cutaneous T-cell lymphoma. Most cases are composed of large anaplastic cells. However, it presents a wide spectrum of histologic patterns. In the small cell variant, a small-sized pleomorphic cell morphology can be seen. A 74-year-old woman presented with an 8-month history of asymptomatic ulcerative plaque and satellite nodule on the right calf. Her past medical history was not specific. The histologic findings on punch biopsy specimens showed a malignant small round cell tumor on both lesions. The tumor cells had large pleomorphic nuclei with multinucleation and some eosinophilic cytoplasm. We performed immunohistochemical staining to rule out neuroectodermal tumor, neuroendocrine tumor, melanoma, lymphoma, and so on. However, the staining results were negative for pancytokeratin, CD3, CD20, CD99, chromogranin A, synaptophysin, CD56, ALK, HMB45, desmin, kappa, lambda, myoglobin, and S-100 protein. CT, MRI, and PET-CT were negative for extracutaneous involvement. Total excision was done, and additional immunohistochemical staining was performed to confirm the origin of the tumor. Staining results for vimentin, LCA, CD4, and CD30 were positive. We concluded that these findings were consistent with the small cell variant CD30+ PCALCL, which occurs rarely.
Aged ; Biopsy ; Chromogranin A ; Cytoplasm ; Desmin ; Eosinophils ; Female ; Humans ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic* ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell, Cutaneous ; Magnetic Resonance Imaging ; Melanoma ; Myoglobin ; Neuroectodermal Tumors ; Neuroendocrine Tumors ; S100 Proteins ; Synaptophysin ; Ulcer ; Vimentin

Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a rare subtype of malignant non-Hodgkin lymphoma, in which 40% of the cases show spontaneous regression without aggressive treatment. Surgery and focal radiation therapy are the primary forms of treatment for this disease; however, if pcALCL is accompanied by multifocal skin lesions, chemotherapy is also common. The prognosis for pcALCL is generally excellent, with a 5-year survival rate of 85-100%. However, pcALCL with extensive limb disease typically has a poor prognosis. Here, we present a case of pcALCL with extensive limb disease that resulted in the patient's death, despite the use of aggressive chemotherapy.
Drug Therapy ; Extremities* ; Lymphoma ; Lymphoma, Non-Hodgkin ; Lymphoma, Primary Cutaneous Anaplastic Large Cell* ; Prognosis ; Skin ; Survival Rate

OBJECTIVETo investigate the frequency of different types of mature T- and NK-cell lymphomas diagnosed in a 4-year period at Sun Yat-sen University Cancer Center, and to study baseline CD30 for potential anti-CD30 targeted therapy in mature T- and NK-cell lymphoma.

METHODSAll cases of mature T- and NK-cell lymphoma diagnosed at Sun Yat-sen University Cancer Center from September 1, 2009 to August 31, 2013, were reviewed. Paraffin-blocks of available 164 consecutive cases were stained for CD30 immunohistochemistry using EnVision protocol.

RESULTSA total of 625 cases of mature T- and NK-cell lymphomas were diagnosed and the most common type was extranodal NK/T cell lymphoma (ENKTL), nasal type 319 (51.0%) cases, followed by angioimmunoblastic T-cell lymphoma (AITL) (119 cases, 19.0%), peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) (81 cases, 13.0%), and anaplastic large-cell lymphoma (ALCL), including 48 cases (7.7%) of systematic ALCL and 11 cases (1.8%) of primary cutaneous ALCL. Besides ALCL, ENKTL had the highest expression rate of CD30 among the 164 cases, with positivity observed in 41 cases (62.1%, 41/66). Only 1 case of PTCL-NOS was CD30 positive. CD30 was not expressed in all 28 cases of AITL and other rare types of mature T- and NK-cell lymphoma.

CONCLUSIONSThe frequency of different types of mature T- and NK-cell lymphoma encountered at Sun Yat-sen University Cancer Center was similar to that seen in other areas of China and other Asia countries. CD30 expression is different among several types of mature T- and NK-cell lymphoma. In addition to ALCL, ENKTL has the highest expression rate of CD30, which may be a candidate disease for anti-CD30 targeted therapy.

China ; epidemiology ; Humans ; Immunohistochemistry ; Killer Cells, Natural ; Lymphoma, Extranodal NK-T-Cell ; epidemiology ; pathology ; Lymphoma, Large-Cell, Anaplastic ; epidemiology ; pathology ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; epidemiology ; pathology ; Lymphoma, T-Cell, Peripheral ; epidemiology ; pathology ; T-Lymphocytes