Severe upper eyelid mass in a patient with mycosis fungoides.

Mycosis fungoides is a cutaneous T-cell lymphoma that has been rarely reported to involve ocular structures. Ophthalmic manifestations usually appear in advanced disease. A case of a 58-year-old man presenting with progressive, full thickness, giant upper eyelid mass is presented. The patient had a long history of recurrent tumoral lesions on the trunk and limbs, previously diagnosed as mycosis fungoides. The histopathological examinations of eyelid tumor supported the diagnosis of mycosis fungoides. The mycosis fungoides was stage as IIB (T3N0M0B0) by TNMB classifications and referred to the Hematology and Radiation Oncology clinics. The importance of ophthalmic involvement is being seen in advanced or refractory cases, and there is a possible relation between mycosis fungoides and poor prognosis by being an early indicator of systemic involvement.

Solitary mycosis fungoides: a distinct clinicopathologic entity with a good prognosis: a series of 15 cases and literature review.

BACKGROUND: Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma (CTCL), accounting for almost 50% of all primary cutaneous lymphomas. The occurrence of solitary lesions, which are clinically and histopathologically indistinguishable from classic MF has been described. OBJECTIVE: We describe 15 cases of solitary MF and discuss the relationship to classic MF, "reactive" processes and to other, rarer forms of CTCL that may present with solitary lesions. METHODS: We conducted a retrospective chart review and a PubMed search to identify all reported cases of solitary MF to date, as well as information about other CTCLs presenting as a solitary lesion. RESULTS: Fifteen patients were identified. Follow-up data were available on 10 patients with a median follow-up of 10 months (range, 1 to 48 months). Clinical, pathological, immunocytochemical, and molecular-genetic features were analyzed. Five cases were diagnosed as folliculotropic MF (FMF). Of the 10 cases with follow-up, 2 were treated with topical steroids, 2 were completely excised, 5 received radiotherapy, and 1 received tacrolimus. One hundred twenty-eight cases of solitary MF were identified in the literature and reviewed for commonalities to and differences with our cases and other CTCLs. LIMITATIONS: This study was retrospective; follow-up data were not available in some cases and were only short term in others. CONCLUSIONS: Solitary MF appears to have a good prognosis. In lesions that are not completely excised, curative radiotherapy can be used. Long-term follow up is advised.

Mycosis Fungoides of the True Vocal Folds: A Case Report.

OBJECTIVES: To report a case of laryngeal involvement of mycosis fungoides and its symptomatic treatment with laser-assisted surgical ablation. METHODS: Case report and literature review. RESULTS: A 76-year-old woman with longstanding MF previously treated with Brentuximab Vedotin who developed persistent cough and dysphonia. The patient's laryngeal disease burden was treated with KTP-laser ablation and further reduced with doxorubicin and radiotherapy. CONCLUSIONS: Although laryngeal, and especially glottic, involvement is a rare finding, suspicion should be maintained in symptomatic patients with cutaneous mycosis fungoides. This the first reported surgical laser treatment of laryngeal symptoms in this context, which can greatly improve a patient's quality of life.

[Long-term efficacy of autologous stem cell transplantation for stage IV mycosis fungoides]. / Efficacité a long terme d'une autogreffe de cellules souches au cours d'un mycosis fongoïde de stade IV.

BACKGROUND: Mycosis fungoides during large cell transformation to lymphoma has a poor prognosis with mean survival of 36 months. Autologous stem cell transplantation is rarely proposed in this indication. We report the case of a young man still in complete remission for transformed mycosis fungoides 14 years after autologous stem cell transplantation. CASE REPORT: A 25-year-old man presenting eczema-like patches since childhood was treated by chemotherapy for multiple lymphadenopathies considered as Hodgkin's lymphoma. He was referred with diffuse skin tumours and infiltrated patches. Histology of tumour samples revealed atypical T-cell infiltrate with epidermotropism and presence of more than 25% of large CD30-positive cells. Non-infiltrated patches showed small T-cell lymphoma with epidermotropism. Histological verification of a previous lymphadenopathy confirmed the diagnosis of transformed mycosis fungoides. Despite multiple courses of chemotherapy, the disease progressed, with neurological involvement in particular. Because of tumour aggressiveness, autologous stem cell transplantation was performed and resulted in rapid regression of the tumours, lymphadenopathy and neurological symptoms. Non-transformed mycosis fungoides patches persisted but were controlled with topical mechlorethamine. This patient is still in complete remission for tumour and extracutaneous lesions 14 years after the autograft. DISCUSSION: This was probably a case of juvenile mycosis fungoides diagnosed and transformed in adult age. Neurological involvement by mycosis fungoides is rare and usually carries a drastic prognosis. To our knowledge, this is the longest remission of transformed mycosis fungoides seen after autograft. It highlights the value of this method in aggressive transformed mycosis fungoides, especially in patients ineligible for allograft.

Two cases of phenotypic switch of primary cutaneous T cell lymphoma after treatment with an aggressive course and review of the literature.

A "phenotypic switch" (PS) is a well-known phenomenon that occurs in hematopoietic neoplasms, often after treatment. However, in cutaneous T cell lymphoma (CTCL), this event has rarely been reported, and thus, very little is known about its relevance to disease prognosis. We report two cases of patients that were diagnosed with a CD4+ mycosis fungoides with positive T cell receptor gene rearrangement studies. Both patients originally responded to treatment, but subsequently, their CTCL came back with a different phenotype of a CD4- CTCL. Gene rearrangement studies were performed on the second occurrence in order to prove that this was the same lymphoma. Both patients died from their CTCL. Additionally, we collected seven cases of primary CTCL from the literature with tissue samples from before and after treatment with molecular studies confirming these neoplasms contained the same T cell clone, providing evidence of a true PS. This too revealed a poor prognosis in the majority of these cases. CTCL should be worked up to determine whether a PS has occurred after therapy since it could confuse management of patients and appears to portend a poor prognosis.

Stem cell transplantation in advanced cutaneous T-cell lymphoma.

Cutaneous T-cell lymphomas are a type of indolent non-Hodgkin's lymphoma where patients with limited skin disease can be successfully treated with a variety of skin-directed, systemic, and immunomodulating therapies, whereas durable remissions are difficult to achieve in patients with tumor, erythrodermic, or systemic disease. We describe a patient with cutaneous T-cell lymphoma and malignant cells constituting 99% of her peripheral blood lymphocytes who had a sustained complete response after an HLA-matched sibling allogeneic stem cell transplantation. We also review the current literature regarding both autologous and allogeneic stem cell transplantations for advanced stages of cutaneous T-cell lymphoma, discuss the importance of the graft-versus-tumor immunomodulatory effect in successful transplantations, and suggest that allogeneic stem cell transplantation deserves further consideration and study as a potential treatment for selected patients who are younger and at high risk.

Atypical fibroxanthoma presenting in a patient with stage III mycosis fungoides.

The association of atypical fibroxanthoma (AFX) and mycosis fungoides (MF) has never been reported. We report a 77-year-old female who was referred for Mohs micrographic surgery (MMS) of an AFX on the right hand. At the time of presentation, this patient had stage III MF, with erythroderma and palpable lymph nodes. Our patient's AFX measured 1.5x2.8 cm. The tumor was removed by MMS with clear margins. The pathologic specimen revealed a relatively well-circumscribed neoplasm in the upper dermis composed of atypical spindle cells with pleomorphism, hyperchromatic nuclei, and brisk mitotic activity. Immunohistochemical stains were focally positive for CD68, and negative for S-100, Melan-A, desmin, smooth muscle actin and neurofilament. Perilesional skin revealed epidermal hyperplasia, parakeratosis, and multiple epidermotropic lymphocytes with enlarged and hyperchromatic nuclei. One year after the initial MMS, our patient developed another tumor on the right hand, which measured 4x6 mm. This neoplasm underwent a fusiform excision with clear margins. Histological examination revealed evidence of MF and an AFX. To our knowledge, this is the first report of an AFX arising in a patient with MF.

[Successful use of allogeneic stem cell transplantation for treatment-refractory mycosis fungoides]. / Erfolgreicher Einsatz einer allogenen Stammzelltransplantation bei therapierefraktärer Mycosis fungoides.

Mycosis fungoides is the most common type of cutaneous T-cell lymphoma and characterized by a chronic progressive course spanning decades. The choice of treatment options should be tailored to the stage depending on the extent and aggressiveness of the disease and taking the individual situation of the patient into consideration. Long-term complete remissions can only be achieved in the early phase of the disease, while there is no therapy that results in a cure or long-term remission in advanced stages. In young patients with a treatment-refractory course of mycosis fungoides, allogeneic stem cell transplantation represents an important alternative option to manage the disease since complete clinical remission can be obtained even in advanced stages.

Mycosis fungoides - A cutaneous lymphoproliferative disorder in a patient treated with fingolimod for multiple sclerosis.

Fingolimod was the first oral disease-modifying drug approved for the treatment of relapsing-remitting multiple sclerosis (MS). It has previously been associated with rare cases of lymphoma. Here we describe the first case of mycosis fungoides - a cutaneous lymphoproliferative disorder, in an MS patient treated with fingolimod. who developed histologically confirmed mycosis fungoides 3 years after starting fingolimod. The drug was withdrawn and the patient was treated with radiotherapy and surgical excision with remission. This report points to a possible association between fingolimod and skin lymphoproliferative disorder and emphasizes the need for periodic skin examination.

[Treatment of mycosis fungoides by 308 nm excimer laser: a clinical and histological study in 10 patients]. / Traitement du mycosis fongoïde par le laser excimer à 308 nm: étude anatomo-clinique chez dix malades.

BACKGROUND: Mycosis fungoides is a cutaneous T-cell lymphoma. The early stages have a good prognosis but pose therapeutic problems due to chronic disease status and the frequent recurrence of lesions. We performed a retrospective clinical and histological study in 10 patients presenting mycosis fungoides in order to evaluate the efficacy of 308 nm excimer laser in this indication. METHODS: Ten patients with mycosis fungoides confirmed by histological examination were included. Treatment by 308 nm excimer laser was performed on a total of 29 lesions (25 patch-stage, 3 plaques and 1 nodule). After determination of the minimal erythemal dose, sessions were started twice weekly gradually increasing in accordance with tolerability. Clinical follow-up was performed and histological and immunohistological analysis was done, if possible, before and after treatment and at the end of the follow-up. RESULTS: Eighty-six percent of patch-stage or plaque lesions had completely cleared up by the end of treatment and 14% had cleared up partially, with a global response rate of 100%. The mean number of sessions was 15 (6 to 46 sessions) corresponding to a mean treatment duration of 2 months. The mean cumulative dose was 5 J/cm2 (1.3 to 16.1 cm2). Follow-up was performed in 19 lesions for a median period of 15 months (8 to 26 months). Persistent complete clearance was observed in 13 of 19 (68%) lesions (12 patch-stage and 1 plaque). Continued partial clearance was noted in 3 lesions (1 patch-stage and 2 plaques) (16%). Two lesions (both patch-stage) (11%) showed relapse 7 months after the end of treatment. The sole nodule in the study showed no response to treatment. Histological and immunohistological were consistent with clinical results and showed histological healing where lesions had clinically disappeared, except for one case with persistence of a few mycosis cells in the epidermis despite a clinical appearance of healing. DISCUSSION: This study demonstrates the efficacy of 308 nm excimer laser in stage Ia of mycosis fungoides. Given its limited availability and high cost, 308 nm excimer laser may be used as second-line therapy after failure of treatment with topical steroids.

[Transformed juvenile-onset mycosis fungoides: treatment by bone marrow transplantation with graft-versus-lymphoma effect]. / Mycosis fongoïde transformé chez un enfant: traitement par transplantation médullaire allogénique avec effet "graft-versus-lymphoma"

BACKGROUND: Mycosis fungoides is the most frequent cutaneous T-cell lymphoma but has been rarely reported in children and teenagers. Although transformation into large-cell T-cell lymphoma has been described in 10% of adult cases, it is seen very rarely in children. We report here the clinical case of mycosis fungoides in a child with an unusual transformed form at presentation and treated by bone marrow allograft. CASE-REPORT: A 13 year-old boy, presenting guttate parapsoriasis for 5 years was referred to our Dermatology Department with a 2-month history of infiltrated plaques throughout the body and face. Large erythematous-squamous plaques on the trunk and face as well as a nodular lesion of the arm were also noted. On histology, typical features of mycosis fungoides were observed, in addition to transformed cells which were CD30-negative. Local treatment comprising caryolysin and dermal corticosteroids allowed initial regression of the lesions. However, a few months later, nodular lesions reappeared as well as axillary lymph nodes. Repeated histology confirmed the diagnosis of transformed mycosis fungoides with large CD30-positive cells. Despite chemotherapy, cutaneous and lymph node disease recurred, and bone marrow allograft was performed, resulting in rapid disease regression. Following the recurrence of skin lesions 2 years later, donor lymphocytes were administered in addition to treatment with interferon alpha, aiming at stimulating a graft-versus-lymphoma reaction. One year post-lymphocyte injection, the patient is in full remission. DISCUSSION: This is a new case report of juvenile mycosis fungoides with unusual clinical features such as rapid course and transformed form at presentation. Juvenile mycosis fungoides represents 2.5 to 5% of cases of mycosis fungoides and transformation to large cell lymphoma is exceptional. Our case illustrates the aggressive pattern observed in some teenage patients as well as the efficacy of bone marrow allograft, most likely thanks chiefly to its graft-versus-lymphoma effect.

Epitheliotropic lymphoma in a squirrel (Sciurus sp.).

A 12-yr-old, intact male squirrel (Sciurus sp.) presented with a 15 mm-by-20 mm area of alopecia and plaque-like dermal thickening over the left caudolateral thorax. Routine diagnostic tests ruled out more common conditions that result in alopecia, such as dermatophytosis and acariasis. A punch biopsy was obtained under anesthesia and submitted for histopathologic evaluation. The diagnosis of epitheliotropic lymphoma was made, and follow-up surgical excision was performed. Histopathologic features were consistent with epitheliotropic lymphoma, and immunohistochemistry confirmed a T-cell origin. There was no local recurrence, new lesions, or evidence of metastasis 10 mo after surgical excision. To our knowledge, to date, epitheliotropic lymphoma has not been described in a squirrel.

[Is it possible to cure a patient with mycosis fungoides? A case report]. / Es posible curar a un paciente con micosis fungoides? Informe de un caso.

Sezary syndrome (SS) and mycosis fungoides (MF) are a group of non Hodgkin lymphomas that originate from T-lymphocytes and involve mostly the skin. These entities are generally non treatable and patient prognosis remains poor even with the advent of current treatment schedules. Complete remissions are seldom observed. For this reason, bone marrow transplant has been used as a treatment option. The high mortality associated with this procedure has turned reduced intensity conditioning stem cell transplant into a treatment option. This case study illustrates how stem cell transplant offers complete remission of this type of lymphomas.

Surgical Excision of Mycosis Fungoides Using Thumb-Sparing Reconstruction.

Background: The differential for soft tissue tumors of the hand and upper limb is broad. Hematologic malignancy remains quite low on the differential for soft tissue tumors involving the hand, and there is little in the literature describing surgical management of such cutaneous manifestations. When the tumor is large or involves the thumb, careful consideration of reconstructive options is required. Methods: We present a rare case of an aggressively enlarging mycosis fungoides, a cutaneous T-cell lymphoma tumor, involving the thumb. This tumor had a history of multiple failed treatment attempts, including radiation and chemotherapy. Results: Our surgical plan was a reverse radial forearm osteocutaneous flap. Conclusion: A reverse radial osteocutaneous forearm flap was successfully used to avoid thumb amputation and preserve thumb function.

Perineural and intraneural cutaneous granulomas in granulomatous mycosis fungoides mimicking tuberculoid leprosy.

BACKGROUND: Histological similarities between granulomas and granulomatous mycosis fungoides (GMF) may lead to misdiagnoses of sarcoidosis or leprosy. METHODS: This report presents four patients with GMF in whom skin biopsies showed perineural and intraneural granulomas that were confused with tuberculoid leprosy granulomas. RESULTS: Patient 1 presented with erythematous plaques and bulky nodules. Biopsy findings suggested cutaneous sarcoidosis. Tumor resection showed granulomatous infiltrate extending to the fascia and skeletal muscle. Clinicopathological correlations permitted a diagnosis of GMF. Patient 2 presented with erythematous plaques. Skin biopsies had indicated sarcoidosis. Resection of a thigh nodule excluded leprosy, and GMF was diagnosed. Patient 3 presented with scaly, hyperpigmented plaques. Biopsy showed diffuse granulomatous inflammation with epithelioid and giant cells, abundant lymphocytes, and some eosinophils, and indicated GMF. Patient 4 presented with pruritic, erythematous plaques. Biopsy of an indurated mammary plaque initially indicated sarcoid granulomatous inflammation. Biopsy review suggested GMF. CONCLUSIONS: This study highlights both the diagnosis of GMF, and granulomatous cutaneous nerve injury in GMF and its possible confusion with leprosy granulomas. The histological diagnosis of GMF includes: (i) a granulomatous infiltrate rich in giant cells, emperipolesis, histiocytic cells, and scattered eosinophils, which may reach the fascia and muscle; (ii) the absence of elastic fibers or their phagocytosis by giant cells; and (iii) lymphocytes that may show atypia and epidermotropism. Deep biopsies reveal GMF diagnostic changes and, in conjunction with clinicopathological correlations, exclude a diagnosis of leprosy and support one of GMF, thus facilitating its appropriate management.

Hematopoietic Stem Cell Transplant for Mycosis Fungoides and Sézary Syndrome.

Mycosis fungoides (MF) and Sézary syndrome (SS) are common types of primary cutaneous T-cell lymphoma. Early-stage MF has a favorable prognosis and responds well to skin-directed regimens. Patients with advanced-stage MF, transformed MF, and SS are treated with combined systemic and skin-directed therapies. However, the disease is incurable with standard regimens, and frequent relapses are common. Owing to the lack of improvement in overall survival with standard regimens, hematopoietic stem cell transplant (HSCT) has been explored as a potential curative option. This article reviews the role of HSCT in MF/SS and discusses data regarding conditioning regimens, treatment-related complications, and outcomes.

Autologous bone marrow transplantation for advanced stage mycosis fungoides.

Patients with advanced stage cutaneous T cell lymphoma (CTCL) have a median survival of 2-5 years with no currently available curative therapy. This limited pilot study was performed to determine if CTCL patients could undergo autologous bone marrow transplantation (ABMT) as a curative treatment without developing life-threatening infections. Since selection of a chemotherapeutic regimen is essentially empirical at this time, several drug combinations were screened. Total skin electron beam radiotherapy was used prior to transplantation to control the skin disease of four patients. Six patients have been transplanted and all have engrafted normally. Infections that developed after transplantation responded to conventional therapy and were typical of those observed in other patients undergoing ABMT. Five of the six patients had a complete clinical response to ABMT but three of these responses lasted less than 100 days. Two recent patients who were treated with carmustine, etoposide, and cisplatin are alive more than 1 year after transplantation without evidence of active disease. Thus, although this study does not prove the efficacy of ABMT, it does demonstrate that patients with CTCL can undergo ABMT without developing life-threatening infections and that carmustine-etoposide-cisplatin plus ABMT should be evaluated in subsequent studies to treat patients with poor prognosis CTCL.

Mycosis fungoides metastasizing to the brain parenchyma: case report.

OBJECTIVE AND IMPORTANCE: Mycosis fungoides is a rare T-cell lymphoma of the skin that can, in one-half to three-quarters of patients suffering from this disease, involve the viscera in late stages of the disease. Although autopsy series performed more than 2 decades ago showed that the incidence of metastatic mycosis fungoides to the central nervous system is approximately one of seven, a total of only several dozen cases have been reported to date. As compared to meningeal involvement, intraparenchymal metastases are even rarer. We describe a biopsy-proven case of intraparenchymal central nervous system mycosis fungoides in a patient with nonprogressive skin involvement and no detectable visceral involvement, and we present a review of the relevant literature. CLINICAL PRESENTATION: A 68-year-old man, 3 years after the diagnosis of his skin disease, developed fatigue, confusion, and frontal lobe signs without the presence of cerebriform cells in the peripheral blood or any other clinical evidence of visceral involvement. Magnetic resonance imaging revealed a diffuse area of increased T2-weighted signal involving the white matter of both cerebral hemispheres as well as a focal area of T2 abnormality along the body of the corpus callosum. The radiological differential diagnosis was either leukodystrophy caused by chemotherapy, progressive multifocal leukoencephalopathy, or glioma with associated white matter changes. INTERVENTION: A stereotactic serial brain biopsy revealed diffuse perivascular infiltrates of atypical lymphocytes, as well as several large cells with cerebriform nuclei consistent with mycosis fungoides. The cells were immunoreactive for LCA, MT1, UCHL1, and CD3. CONCLUSION: We stress the importance of including mycosis fungoides as part of the differential diagnosis for a brain lesion in patients with cutaneous T-cell lymphoma, because treatments do exist, and we conclude that a serial stereotactic biopsy may be necessary to provide a definitive diagnosis.

[Extreme soft tissue hypertrophy of both hands of "uncertain origin"]. / Extreme Weichteilhypertrophie beider Hände "unklarer Genese".

The author reports on a 56-year old patient. At the age of 17 an asymmetric hypertrophy of the soft tissue isolated on both hands developed increasingly. Based upon differing diagnoses, corticoids and "black light - phototherapy" were attempted. In 1985 and 1986 surgery of both hands was performed. Finally in 1987, after 38 years, "Mycosis fungoides phase III" of the hands was diagnosed and adequately treated.