Primary cutaneous anaplastic large cell lymphoma of the face presenting as posttraumatic maxillary sinusitis.

Primary cutaneous anaplastic large cell lymphoma of the face presenting as posttraumatic maxillary sinusitis.
J Craniofac Surg. 2008 Nov

Chen RF, Chen CT, Liao HT, Chen CH, Chen YR.
Division of Trauma Plastic Surgery, Department of Plastic and Reconstruction Surgery, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Chang Gung Institute of Technology, Taoyuan, Taiwan.

Primary cutaneous anaplastic large cell lymphoma (ALCL) is an uncommon disease. It has various presentations that may mislead the diagnosis and cause delay of treatment. We report a 12-year-old boy who experienced persistent swollen and painful right cheek after blunt facial injury. The patient was first treated for maxillary sinusitis according to clinical history, but the pathology turned out to be primary cutaneous ALCL. Symptoms improved after chemotherapy with complete remission. No recurrence was noted during the follow-up period. The presentations of primary cutaneous ALCL of the face may mimic posttraumatic maxillary sinusitis. Any patient with facial prolonged symptoms or nonhealing wound should be excluded from this entity.

Lippincott, Williams & Wilkins

Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma.

Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma.
Arch Dermatol. 2008 Jun

Gerami P, Rosen S, Kuzel T, Boone SL, Guitart J.
Department of Dermatology, Northwestern University Feinberg School of Medicine, and The Robert H. Lurie Comprehensive Cancer Center, Northwestern University, Chicago, Illinois, USA.

OBJECTIVES: To study the clinical features, therapeutic responses, and outcomes in patients with folliculotropic mycosis fungoides (FMF) and to compare our single-center experience of 43 patients with the findings from the Dutch Cutaneous Lymphoma Group.

SETTING: A single-center experience from the Northwestern University Multidisciplinary Cutaneous Lymphoma Group.

PATIENTS: Forty-three patients with FMF were included in the study and compared with 43 age- and stage-matched patients with classic epidermotropic mycosis fungoides (MF) with similar follow-up time.

RESULTS: Folliculotropic mycosis fungoides showed distinct clinical features, with 37 patients having facial involvement (86%) and only 6 having lesions limited to the torso (14%). The morphologic spectrum of lesions is broad and includes erythematous papules and plaques with follicular prominence with or without alopecia; comedonal, acneiform, and cystic lesions; alopecic patches with or without scarring; and nodular and prurigolike lesions. Sixty-five percent of patients had alopecia, which in 71% of cases involved the face. Severe pruritus was seen in 68% of patients. In general, patients responded poorly to skin-directed therapy and in almost all cases required systemic agents to induce even a partial remission, including patients with early-stage disease. Overall survival was poor. Patients with early-stage disease (< or ="IIA)"> or =IIB) had an outcome similar to those patients in the control group with conventional epidermotropic MF of a similar stage.

CONCLUSIONS: The morphologic spectrum of clinical presentation for FMF is broad and distinct from those in conventional MF. This is at least partially attributed to the ability of FMF to simulate a variety of inflammatory conditions afflicting the follicular unit. The disease course is aggressive, and many patients, including those with early disease, show a poor outcome particularly between 10 and 15 years after the initial onset of disease. Response to skin-directed therapy is poor even in early-stage disease, and our best results were seen with psoralen plus UV-A (PUVA) therapy with oral bexarotene or PUVA with interferon alfa. These findings corroborate those of the Dutch Cutaneous Lymphoma Group and further validate the classification of FMF as a distinct entity.

PubMed

A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy.

A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy.
J Am Acad Dermatol. 2009 Jan

Jacob R, Scala M, Fung MA.
Department of Radiation Oncology, University of California-Davis, Sacramento, California, USA.

Syringotropic cutaneous T-cell lymphoma (SCTCL) is a variant of mycosis fungoides that is characterized by the presence of lymphocytic infiltration of eccrine glandular structures and the absence of the classic features of MF, such as epidermotropism and Pautrier's microabscesses. We report a patient with SCTCL who presented with multiple hypopigmented patches and localized alopecia who was treated with local radiotherapy with excellent local control. In our experience, local electron radiation offers local control in early stage SCTCL, and it may be possible to reserve systemic therapy for more advanced stages of the disease.

PMID: 19103368 [PubMed - in process]

Primary cutaneous T-cell-rich B-cell lymphoma: a case report and literature review.

Primary cutaneous T-cell-rich B-cell lymphoma: a case report and literature review.
Acta Dermatovenerol Alp Panonica Adriat. 2008 Dec

Venizelos ID, Tatsiou ZA, Mandala E.
Pathology Department, Hippokration Hospital, 5 Exadactylou Street, 54635 Thessaloniki, Greece. ivenizel@otenet.gr.

T-cell-rich B-cell lymphoma (TCRBCL) is a recently recognized B-cell lymphoma variant, characterized by a minor population of neoplastic B-cells existing in a background of predominant reactive T-lymphocytes. It is a rare entity, accounting for approximately 1 to 2% of all non-Hodgkin's lymphomas. It has both nodal and extranodal presentation. Primary cutaneous TCRBCL is an extremely rare lymphoma and only 16 cases have been documented thus far in the medical literature. We report the case of a 46- year-old man that presented with a slowly-growing, painless skin nodule on the left temporofrontal region of the scalp. A complete surgical excision was performed and histological examination revealed diffuse infiltration of the dermis by TCRBCL. A complete surgical excision of the skin lesion and systemic chemotherapy seems to have been effective because the patient is disease-free 2 years after the initial diagnosis was made. This study reports a very rare case of TCRBCL presented primarily in the skin. Because of its rarity, it is especially important to make the correct diagnosis using the appropriate immunohistochemical stains and apply the proper therapy.

PMID: 19104743 [PubMed - in process]

Lymphomatoid papulosis in childhood: six case reports and a literature review

Lymphomatoid papulosis in childhood: six case reports and a literature review
Ann Dermatol Venereol. 2008 Oct

Bories N, Thomas L, Phan A, Balme B, Salameire D, Thurot-Guillou C, Dalle S.

Service de dermatologie, hôpital Hôtel-Dieu, 1 place de l'Hôpital, Lyon cedex 02, France.

BACKGROUND: Lymphomatoid papulosis (LyP) is a rare lymphoproliferative disorder. It is now included in the World Health Organisation (WHO) classification of cutaneous lymphomas. Although frequently described in adults, there have been only a few reported cases of LyP in children; diagnosis is often difficult in this population and no clear guidelines have been established regarding management or monitoring. In this article we report six new cases of LyP in children.

PATIENTS AND METHODS: This is a retrospective study of six children, aged between two and 11 years, seen at the Hôtel-Dieu Hospital in Lyon and at the Grenoble Hospital Centre between 2005 and 2008. Each child underwent skin biopsy for histological and immunohistochemical analysis.

RESULTS: All six children presented papulonodular lesions on the limbs and trunk, in some cases necrotic, present for different times and developing in episodes. Histology and immunolabelling announces in all six cases militated in favour of LyP type A, with large CD30+ and CD15- cells dispersed in an inflammatory dermal infiltrate made up for the most part of lymphocytes, polynuclear neutrophils, eosinophils and a small number of histiocytes.

DISCUSSION: Only around 60 cases of LyP have so far been reported in children, principally type A. Association with malignant lymphoma occurs, with high risk in relation to the general population of the same age. Clinical diagnosis is confirmed histopathology and immunolabelling. There is currently no consensus regarding therapeutic management. First-line treatment generally comprises therapeutic abstention or dermal corticosteroids. Methotrexate and phototherapy constitute possible alternatives but should be used only in very disseminate or debilitating forms of the disease.

CONCLUSION: The presentation and course of LyP of childhood differs very little from the adult form. Cases of associated lymphoma have been reported. Although regular clinical monitoring is recommended, there is no call for routine laboratory testing.

EMConsulte

Primary cutaneous B cell lymphoma presenting as recurrent eyelid swelling.

Primary cutaneous B cell lymphoma presenting as recurrent eyelid swelling.
Clin Experiment Ophthalmol. 2008 Oct

Pandya VB, Conway RM, Taylor SF.
University of Sydney, Sydney, NSW, Australia.

Primary cutaneous lymphoma represents a distinct clinical entity within the spectrum of haematological malignancy. A case of primary cutaneous B cell lymphoma is reported, presenting in an 87-year-old female with a 2-year history of intermittent swelling and discolouration of the right upper and lower eyelids, in the absence of systemic symptoms. Histopathological examination of an incision biopsy revealed a lymphoid infiltrate in the dermis with immunophenotypic features of B cell lymphoma. Staging investigations confirmed the absence of systemic disease. Treatment with oral chemotherapy was undertaken with a good response. Ophthalmologists should include primary cutaneous lymphoma in the differential diagnosis of recurrent eyelid swelling.

Wiley InterScience

Coincidence of cutaneous follicle center lymphoma and diffuse large B-cell lymphoma.

Coincidence of cutaneous follicle center lymphoma and diffuse large B-cell lymphoma.
Int J Dermatol. 2008 Nov

van Tuyll van Serooskerken AM, Mosterd K, Veraart JC, Vermeer MH, Frank J, van Marion AM.

Department of Dermatology, University Medical Center Maastricht, The Netherlands. atv@sder.azm.nl

Primary cutaneous follicle center lymphoma (PCFCL) is a neoplasm with differentiation of centrocytes and centroblasts presenting in the skin. At the time of initial manifestation, extracutaneous involvement is absent. PCFCL is considered as an indolent variant of primary cutaneous B-cell lymphomas since dissemination to extracutaneous sites is rare and the prognosis is favorable. Here we describe a 30-year-old man who was diagnosed with a cutaneous FCL and did not show extracutaneous affection at the time of occurrence. Six months later, however, he developed a diffuse large B-cell non-Hodgkin lymphoma localized in several lymph nodes of the neck that most likely reflects the occurrence of a second primary tumor in the same patient.

PubMed

Primary cutaneous aspergillosis in a patient with cutaneous T-cell lymphoma.

Primary cutaneous aspergillosis in a patient with cutaneous T-cell lymphoma.
Mycoses. 2008 Oct 18

Yuanjie Z, Jingxia D, Hai W, Jianghan C, Julin G.
Department of Dermatology and Mycology Center, Changzheng Hospital, Second Military Medical University, Shanghai, China.

We present a case of primary cutaneous aspergillosis caused by Aspergillus terreus in a patient with cutaneous T-cell lymphoma. A 41-year-old woman bruised her left shin and presented with a 1 x 2 cm ecchymosis on the upper third of the left shin for over 6 months. Before mycological examination and sequential biopsies, the patient was erroneously treated with antibiotics and local debridement. After final diagnosis of cutaneous aspergillosis caused by A. terreus cutaneous T-cell lymphoma on the basis of mycological, histopathological and immunohistochemical findings, the patient responded well to oral itraconazole and chemotherapy for over 1 month.

PubMed

Cutaneous B-cell lymphoma

Cutaneous B-cell lymphoma

What are cutaneous B-cell lymphomas?

Lymphomas are tumours of the lymph nodes and lymphatic system. Extranodal lymphomas are tumours that occur in organs or tissues outside of the lymphatic system. When lymphomas occur in the skin with no evidence of extracutaneous disease at the time of diagnosis, they are called ‘primary’ cutaneous lymphomas. There are many different types of primary cutaneous lymphomas but they can be broadly divided into two categories, cutaneous T-cell lymphomas and B-cell lymphomas. Primary cutaneous lymphomas of B cell type comprise approximately 20% of cutaneous lymphomas.

Cutaneous B-cell lymphomas occur when there is a malignant proliferation of lymphocytes of the B-cell type. Mutation occurring at different points in B cell development leads to differing forms of lymphoma.

Classification of cutaneous B-cell lymphomas

Recently the World Health Organisation (WHO) and European Organization for Research and Treatment of Cancer Classification (EORTC) reached a consensus classification for cutaneous lymphomas1. Primary cutaneous B-cell lymphomas include:

Primary cutaneous follicle centre lymphoma
Primary cutaneous marginal zone B- cell lymphoma
Primary cutaneous diffuse large B-cell lymphoma, leg type
Primary cutaneous diffuse large B-cell lymphoma, other

Primary cutaneous follicle centre (FC) lymphoma

These skin lymphomas typically present as solitary or grouped nodules, tumours, or infiltrative plaques. They occur most commonly in the scalp, forehead, neck, trunk and back. The tumours have an indolent (slow growing) course but progress with time. Neoplastic follicle centre cells, usually a mixture of centrocytes (small and large cleaved follicle centre cells) and centroblasts (large noncleaved follicle centre cells with prominent nucleoli) are seen in the dermis in at least part of the infiltrate, but usually spare the overlying epidermis. Cellular morphology may vary with the age and size of the lesion, with care required to differentiate this group from Primary cutaneous diffuse large B-cell lymphoma, leg type.

Primary cutaneous FC lymphoma needs to be differentiated from a secondary cutaneous lymphoma, which is where a nodal follicular lymphoma has spread to involve the skin (skin metastasis). It appears from molecular biology studies that Primary cutaneous FC lymphoma usually lack expression of the bcl-2 protein, and, the t(14;18) translocation, which if present may suggest systemic follicular lymphoma.

Primary cutaneous FC lymphomas may be treated with radiotherapy or combination chemotherapy. Radiotherapy is the treatment of choice for localised tumours whereas multiple tumours on the skin of different parts of the body (multifocal) may respond better to combination chemotherapy. Patients with primary cutaneous FC lymphomas have a good overall survival rate. Five-year survival is reported to be more than 95%.

Primary cutaneous marginal zone B-cell lymphoma

These are low-grade malignant B-cell lymphomas of the MALT (mucosa-associated lymphoid tissue) type, that occur in the skin as solitary or multiple red to brownish red papules, plaques, or nodules. They are usually located on the trunk or extremities, especially the arms. Histologically, these lymphomas are characterised by the presence of nodular or diffuse infiltrates with small lymphocytes, marginal zone B cells, lymphoplasmacytoid cells and plasma cells localised in the dermis and subcutaneous fat. This group also includes primary cutaneous immunocytomas.

Primary cutaneous immunocytoma and marginal zone B-cell lymphoma are classed as indolent lymphomas with excellent prognosis. Five-year survival rate is close to 100%. Dissemination to extracutaneous sites is very rare, however there are reports of it spreading to other organs and the bone marrow.

Radiotherapy or surgical excision is effective treatment for patients with a solitary or few lesions. Multifocal skin lesions are treated with chemotherapy agents such as chlorambucil, interferon alpha and anti-CD20 antibody (rituximab). As this is an indolent lymphoma, observation is a reasonable approach in some cases.

Primary cutaneous large B-cell lymphoma, leg type

These cutaneous B-cell lymphomas occur mainly in elderly females and present as erythematous red or bluish-red nodules or tumours usually on one or both lower legs. These tumours are more aggressive than large B-cell lymphomas of the head and neck, often disseminate to extracutaneous sites and have a more unfavourable prognosis.

Histologically these lymphomas show a diffuse monotonous population of centroblasts and immunoblasts. This picture may be seen in lesions not arising on the leg, and these can be classified into this group.

Only some localised small tumours may be treated using radiotherapy. Anthracycline-based chemotherapy is used for most skin tumours or when tumours have spread to other parts of the body. Some cases have shown response to the anti-CD20 antibody rituximab. Death usually occurs with disseminated disease.

Primary cutaneous diffuse large B-cell lymphoma, other

This group contains large B-cell lymphomas arising in the skin, which do not belong to the primary cutaneous follicle centre lymphoma group or large B-cell lymphoma, leg type. These lymphomas commonly present with skin lesions on the head, the trunk or the extremities and have an excellent prognosis.

Intravascular large B-cell lymphoma is a well-defined subtype of large B-cell lymphoma. Malignant B lymphoid cells proliferate within the lumen of small blood vessels, primarily in the skin and central nervous system. Lesions appear as erythematous, tender nodules, tumours, and telangiectases mainly on the trunk and lower legs. Cutaneous lesions may be confused with mycosis fungoides, sarcoidosis, blood vessel tumours, or secondary cutaneous involvement by lymphoma or leukaemia. As the disease progresses secondary organ involvement often occurs. Prognosis is poor, particularly if the disease has spread to other parts of the body.

Article

What is cutaneous T-cell lymphoma?

What is cutaneous T-cell lymphoma?

Cutaneous T-cell lymphoma is a disease in which certain cells of the lymph system (called T-lymphocytes) become cancer (malignant) and affect the skin. Lymphocytes are infection-fighting white blood cells that are made in the bone marrow and by other organs of the lymph system. T-cells are special lymphocytes that help the body's immune system kill bacteria and other harmful things in the body.

The lymph system is part of the immune system and is made up of thin tubes that branch, like blood vessels, into all parts of the body, including the skin. Lymph vessels carry lymph, a colorless, watery fluid that contains lymphocytes. Along the network of vessels are groups of small, bean-shaped organs called lymph nodes. Clusters of lymph nodes are found in the underarm, pelvis, neck, and abdomen. The spleen (an organ in the upper abdomen that makes lymphocytes and filters old blood cells from the blood), the thymus (a small organ beneath the breastbone), and the tonsils (an organ in the throat) are also part of the lymph system.

There are several types of lymphoma. The most common types of lymphoma are called Hodgkin's disease and non-Hodgkin's lymphoma. These types of lymphoma usually start in the lymph nodes and the spleen. See the patient information summaries on adult or childhood non-Hodgkin's lymphoma or adult or childhood Hodgkin's disease for treatment of these cancers.

Cutaneous T-cell lymphoma usually develops slowly over many years. In the early stages, the skin may itch, and dry, dark patches may develop on the skin. As the disease gets worse, tumors may form on the skin, a condition called mycosis fungoides. As more and more of the skin becomes involved, the skin may become infected. The disease can spread to lymph nodes or to other organs in the body, such as the spleen, lungs, or liver. When large numbers of the tumor cells are found in the blood, the condition is called the Sezary syndrome.

If there are symptoms of cutaneous lymphoma, a doctor may remove a growth from the skin and look at it under a microscope. This is called a biopsy.

The chance of recovery (prognosis) and choice of treatment depend on the stage of the cancer (whether it is just in the skin or has spread to other places in the body) and the patient's general state of health.

There are several other types of cancer that start in the skin. The most common are basal cell cancer and squamous cell cancer, which are covered in the PDQ patient information summary on skin cancer. Another type of skin cancer called melanoma is covered in the patient information summary on melanoma. Kaposi's sarcoma, a rare type of cancer that occurs most commonly in patients with the Acquired Immunodeficiency Syndrome (AIDS), also affects the skin. See the PDQ patient information summary on Kaposi's sarcoma for treatment of this cancer. Cancers that start in other parts of the body may also spread (metastasize) to the skin.

Stages of cutaneous T-cell lymphoma

Once cutaneous T-cell lymphoma is found, more tests will be done to find out if cancer cells have spread to other parts of the body. This is called staging. A doctor needs to know the stage of the disease to plan treatment.

The following stages are used for cutaneous T-cell lymphoma:

Stage I

The cancer only affects parts of the skin, which has red, dry, scaly patches, but no tumors. The lymph nodes are not larger than normal.

Stage II

Either of the following may be true: The skin has red, dry, scaly patches, but no tumors. Lymph nodes are larger than normal, but do not contain cancer cells. There are tumors on the skin. The lymph nodes are either normal or are larger than normal, but do not contain cancer cells.

Stage III

Nearly all of the skin is red, dry, and scaly. The lymph nodes are either normal or are larger than normal, but do not contain cancer cells.

Stage IV

The skin is involved, in addition to either of the following:

Cancer cells are found in the lymph nodes.

Cancer has spread to other organs, such as the liver or lung.

Recurrent

Recurrent disease means that the cancer has come back after it has been treated. It may come back where it started or in another part of the body

From: National Cancer Institute - PDQ

For the complete article, including treatment information, please see the link below.

National Cancer Institute