Epidemiological changes in cutaneous lymphomas: an analysis of 8593 patients from the French Cutaneous Lymphoma Registry.

BACKGROUND: Primary cutaneous lymphomas (PCLs) are a heterogeneous group of T-cell (CTCL) and B-cell (CBCL) malignancies. Little is known about their epidemiology at initial presentation in Europe and about potential changes over time. OBJECTIVES: The aim of this retrospective study was to analyse the frequency of PCLs in the French Cutaneous Lymphoma Registry (GFELC) and to describe the demography of patients. METHODS: Patients with a centrally validated diagnosis of primary PCL, diagnosed between 2005 and 2019, were included. RESULTS: The calculated incidence was unprecedently high at 1·06 per 100 000 person-years. The number of included patients increased yearly. Most PCL subtypes were more frequent in male patients, diagnosed at a median age of 60 years. The relative frequency of rare CTCL remained stable, the proportion of classical mycosis fungoides (MF) decreased, and the frequency of its variants (e.g. folliculotropic MF) increased. Similar patterns were observed for CBCL; for example, the proportion of marginal-zone CBCL increased over time. CONCLUSIONS: Changes in PCL frequencies may be explained by the emergence of new diagnostic criteria and better description of the entities in the most recent PCL classification. Moreover, we propose that an algorithm should be developed to confirm the diagnosis of PCL by central validation of the cases.

[Angiomatoid fibrous histiocytoma of the radial pulse groove]. / Histiocytome fibreux angiomatoïde de la gouttière du pouls radial.

BACKGROUND: Angiomatoid fibrous histiocytoma (AFH) is a slowly progressing rare soft-tissue tumour of moderate malignant potential. It is most commonly seen in children and young adults. Clinically, the lesion is easily confused with a haematoma or soft-tissue haemangioma, and the radiological aspects are not specific. PATIENTS AND METHODS: A 16-year-old male patient presented with a nodular lesion situated very close to the right radial artery, vein and nerve and which had been developing for several years. Surgical resection was carried out with sparing of vasculonervous and functional structures. Histological examination revealed a tumour of plurinodular architecture, surrounded by a fibrous pseudocapsule consisting of histiocytoid or fusiform cells in short bundles associated with a mononuclear inflammatory reaction of nodular architecture. The tumour cells expressed the following immunomarkers: desmin, smooth muscle actin, CD99, and epithelial membrane antigen. Fusion transcript EWSR1-ATF1 was found. DISCUSSION: In this case, as occurs in the literature, a diagnosis of AFH was not made on clinical examination or imaging. The enlarged excision normally recommended was greatly restricted in our patient due to the complex localization of the lesion, which was in contact with major anatomical structures. The diagnosis was based on histological examination of the surgical excision and identification of the fusion gene. Long-term follow-up is required to detect local recurrence or metastasis. Management is decided in multidisciplinary meetings.

[Complication of radical lymph node dissection following sentinel lymph node biopsy in patients with melanoma]. / Complications du curage ganglionnaire réalisé après exérèse du ganglion sentinelle dans le mélanome.

OBJECTIVE: To assess the rate of complications of radical lymph node dissection following sentinel lymph node biopsy in patients with malignant melanoma. PATIENTS AND METHODS: A single-centre retrospective study was performed between 2001 and 2009. All melanoma patients who first had a sentinel node biopsy followed by a radical lymph node dissection were included. RESULTS: Seventy-four patients had radical lymph node dissection after confirmation of metastasis in a sentinel node. The sites of the lymphadenectomy were axillary in 51% of patients, groin in 43%, neck in 5% and popliteal in 1%. A nodal metastasis was observed in 12 of 74 lymph node dissections. Median follow-up time was 41.5 months (3-135 months). At least one complication was seen in 47 patients (64%). Early complications were: postoperative infection (n=9; 12%), lymphocele (n=22; 30%), chronic seroma (n=9; 12%), postoperative haematoma (n=6; 8%), and wound separation (n=7; 9%). Late complications were: lymphoedema (n=30; 41%), joint complications (n=10; 14%), fibrous scar (n=11; 15%), chronic pain (n=6; 8%) and hyposensitivity (n=6; 8%). Two patients were rehospitalised for a complication. The complication rate was 53% for axillary location and 81% for inguinal location (P=0.02). CONCLUSION: Radical lymph node dissection performed after positive sentinel node biopsy in melanoma patients is associated with significant morbidity.

[Cryosurgery and photodynamic therapy for the treatment of Paget's disease of the vulva: two cases]. / Traitement de la maladie de Paget vulvaire par cryochirurgie et photothérapie dynamique topique.

BACKGROUND: Surgery is the standard treatment for vulvar Paget's disease, despite its mutilating consequences and the high associated recurrence rate. Alternative treatments such as CO2 laser, application of imiquimod and topical photodynamic therapy (PDT) have recently been proposed. We report on two patients with vulvar Paget's disease treated with combined cryosurgery and PDT. PATIENTS AND METHODS: Case 1: a 40-year-old pregnant woman presented large vulvar lesions suggestive of Paget's disease. After imiquimod treatment failed, three sessions of cryosurgery were performed on all lesions at 5-month intervals, followed by PDT sessions for the residual erythema. Because of recurrent focal lesions, further sessions of localized cryosurgery were performed for 18 months. Case 2: a 61-year-old patient with extensive vulvar Paget's disease relapsed after imiquimod treatment and was treated by cryosurgery and multiple PDT sessions. She was in complete remission 12 months after this treatment. DISCUSSION: Combined cryosurgery and PDT may offer a therapeutic alternative to conventional surgery for the treatment of large or recurrent multifocal vulvar Paget's disease, or in elderly people. Indeed, although palliative, these two non-invasive techniques can be used to treat many lesions in a single session. Recurrences can be treated again using either technique, or both, without any aesthetic or functional consequences.

[Changes in the incidence and mortality rates of malignant melanoma in the Seine-Maritime region over a 20-year period]. / Évolution de l'incidence et de la mortalité du mélanome en Seine-Maritime sur une période de 20ans.

BACKGROUND: The aim of this study was to assess changes in the incidence and mortality rates of malignant melanoma (MM) over a 20-year period in a region without a cancer registry. METHODS: All cases of MM studied were collected retrospectively from the databases of six private and three hospital-based histopathology laboratories in the Seine-Maritime region covering three 24-month periods each 10years apart: 1988-1989, 1998-1999 and 2008-2009, The incidence and mortality rates were estimated based on data provided by French National Institute for Statistics and Economic Studies (Insee) and French National Institute of Health and Medical Research (Inserm). RESULTS: Over the 20-year period, the incidence of MM increased from 8.6 to 21.2/100,000inhabitants per year (+147%, P<0.0001) while the mortality rate rose from 1.3 to 2.8/100,000inhabitants per year (+115%, P=0.0003). The incidence of invasive MM increased by +110%, while the incidence of MM in situ increased by +456%. The incidence and overall mortality rate of invasive MM increased particularly during the first 10-year period: +62% (P<0.0001) and +77% (P=0.01) respectively, and to a much lesser extent during the last 10-year period: +30% (P=0.0007) and +22% (P=0.22) respectively. This slowdown in the incidence of invasive MM and in overall mortality rates was even more pronounced in women over the last 10years (+17 and +9%), whereas these rates continued to increase in men (+49% and +35%, respectively). In contrast, the incidence of MM in situ increased above all during this same period (+257%). CONCLUSION: This study shows that while the incidence and mortality rate of invasive MM has increased little over the last 10years in the Seine-Maritime region, the incidence of MM in situ continues to rise sharply.

[Early onset pediatric sarcoidosis, diagnostic problems]. / Sarcoïdose à début précoce, difficultés diagnostiques en pédiatrie.

Sarcoidosis, a chronic multisystem inflammatory granulomatous disorder of unknown origin, is a rare disease in children. Two distinct clinical presentations of sarcoidosis in childhood are known. Older children usually show multisystem disease, close to the adult manifestation, with lung infiltration and frequent hilar lymphadenopathy. Prior to the age of 5, sarcoidosis reveals more frequently with the classical triad of rash, arthritis, and uveitis. Due to non-specific clinical features and the lack of a specific test, recognizing sarcoidosis can be difficult in the pediatric population. Moreover, unlike in adults, lung involvement is rare in pediatric sarcoidosis. Given the lack of a definitive blood test, the World Association of Sarcoidosis and Other Granulomatous disorders (WASOG) only recommends dosing the serum angiotensin-converting enzyme (ACE). Its level is usually higher in children than in adults, but an increased ACE may help in the diagnosis. The gold standard is a biopsy specimen with typical epithelioid gigantocellular granuloma without caseating necrosis granuloma, after other disorders known to cause granulomatous disease have been reasonably excluded. We report here the case of a 4.5-year-old male with the history of polyarthritis and uveitis, considered first as juvenile rheumatoid arthritis, followed 5 years later by cutaneous involvement, which led to reconsidering the diagnosis. There were no pulmonary clinical findings. Histology provided the diagnosis of sarcoidosis. He then developed dependence on steroids. The lack of the classical triad delayed the diagnosis several years. This case shows the pediatric singularity of sarcoidosis, which needs to be known so that early and appropriate follow-up can be conducted.

[Abrikossoff's tumor: about one case of inter-mammary cutaneous localization and review of literature]. / Tumeur d'Abrikossoff : à propos d'une localisation cutanée du sillon intermammaire et revue de la littérature.

SUBJECT: Our report concerns a large inter-mammary cutaneous Abrikossoff tumor on a 41-year-old female patient, which has prompted us to review the literature. CASE: It was a low pre-sternal skin tumor; CT-Scanning showed a homogeneous mass 5 cm high, 4 cm wide and 4 cm deep. Total tumoral included the right pectoralis major muscle aponeurosis. The skin defect was closed using a V-Y local plasty. Histologic examination revealed no sign of malignity. DISCUSSION: Abrikossoff tumors (or granular cell tumors) are rare ubiquity tumors locating preferably in ENT and oral cavity mucosa. They develop between 20 and 60 and more often concern women. They are overwhelmingly single and benign but on occasion multifocal or malignant. Immunohistochemistry points to a schwannian origin. Treatment is exclusively surgical. Skin closure is by direct suture, skin grafting or local plasty. The use of flaps is not advised as it may delay local recidivation diagnosis. The literature reports one single other case of chest wall location. CONCLUSION: Abrikossoff tumors require complete exeresis and mandatory histological examination into possible signs of malignity. The risk of recidivation warrants long-term surveillance.

[Imatinib-induced DRESS]. / Syndrome d'hypersensibilité médicamenteuse à l'imatinib.

BACKGROUND: Imatinib (Glivec) is a tyrosine kinase inhibitor used to treat certain cases of leukaemia. We report a case of a drug-induced reaction with eosinophilia and systemic symptoms (DRESS) caused by imatinib. CASE-REPORT: A 77-year-old woman with a chronic myeloid leukaemia was treated with imatinib and allopurinol. Nineteen days after the start of treatment, the patient presented fever with a generalized polymorphous rash associated with oral erosions, facial oedema, diffuse lymphadenopathy and blood hypereosinophilia. Histological analysis of skin biopsy specimens suggested a drug-induced reaction. The outcome was favourable two weeks after discontinuation of treatment. Three months later, imatinib was reintroduced because of progression of the patient's chronic myeloid leukaemia, and recurrence of the skin rash and fever was observed within 12 hours. DISCUSSION: Allopurinol was stopped definitively because of its more frequent imputability. Imatinib was reintroduced after considering the benefit-risk ratio and in full knowledge of the existence of cutaneous reactions to imatinib, despite there being only one recent report of DRESS following treatment with imatinib. According to the causality criteria of Bégaud et al. regarding imatinib, inherent causality of the drug in our patient was initially possible (I2) and appeared likely (I3) after the rechallenge test. This case clearly illustrates that imatinib is a potential cause of DRESS.

[Mucosal involvement in lymphomatoid papulosis: four cases]. / Atteinte muqueuse dans la papulose lymphomatoïde: quatre cas.

BACKGROUND: Lymphomatoid papulosis is a form of CD30+ cutaneous lymphoproliferation characterized by a benign chronic papulonodular eruption that regresses spontaneously. The clinical features contrast with the malignant histological aspect of lesions. Mucosal lesions are rare, with less than 10 published cases. We report four new cases and we highlight characteristic features of lesions at this particular site. PATIENTS AND METHODS: We report four cases of mucous lymphomatoid papulosis in three women aged 37, 38, and 71 years and one 66-year-old man. These cases were collated from three different hospitals: Orléans, Rouen and Caen. Mucosal lesions occurred after cutaneous eruption in two cases but remained isolated or preceded cutaneous lesions in the other two cases. The main site was the mouth in all four cases but one case also involved genital lesions. Two cases involved type A pathological features and two had type C features. Association with lymphoma was excluded on clinical, laboratory and radiological examination. One patient was treated with methotrexate (>7.5mg/week) and did not relapse. Of the three other untreated patients only one did not relapse (short 14-month follow-up). DISCUSSION: Recurrent oral ulcerations may be mucosal manifestations of lymphomatoid papulosis. This site does not appear to have any bearing on prognosis.

[Lymphangiosarcoma treated with liposomal doxorubicin (Caelyx)]. / Lymphangiosarcome traité par doxorubicine liposomale (Caelyx).

BACKGROUND: Lymphangiosarcoma is a highly malignant tumor with a poor prognosis. Anthracyclines constitute the form of chemotherapy most commonly used in these patients. Unfortunately, they are poorly tolerated. We report a case of lymphangiosarcoma in an elderly woman with good response to liposomal doxorubicin, an anthracycline with lower toxicity. CASE REPORT: A 70 year-old-woman with a previous history of post-mastectomy lymphedema presented a painful and bleeding lymphangiosarcoma on the arm and the chest. Because of the wide extent of the tumor, surgery was not performed. The patient was treated with liposomal doxorubicin 50 mg/m2. Marked tumor regression was observed after the first course of chemotherapy. After 5 courses, 90% regression of tumor mass was seen. Pain and bleeding also stopped. Two months after the final course of liposomal doxorubicin, relapse occurred and the patient died. DISCUSSION: A dramatic response to liposomal doxorubicin was noted in the present case, as previously reported in a patient with an angiosarcoma of the scalp. Liposomal doxorubicin could be considered for the treatment of lymphangiosarcoma, particularly in elderly patients.

[Complications after sentinel lymph node excision in patients with malignant melanoma]. / Complications liées à l'exérèse du ganglion sentinelle dans le mélanome.

INTRODUCTION: Side-effects occurring after sentinel lymph node excision in malignant melanoma patients have been poorly evaluated to date. The aim of the present study was to assess the side-effects of sentinel lymph node excision in this population. PATIENTS AND METHODS: All consecutive malignant melanoma patients undergoing sentinel lymph node excision between March 2000 and December 2002 were included in this retrospective study. Patients with a metastatic sentinel node subsequently undergoing lymph node dissection were excluded. Median follow-up of patients was 12.6 +/- 8.8 months. Complications were classified as "early" (i.e. occurring the month following surgery), or "late" (after this time). RESULTS: Forty malignant melanoma patients (17 males, 23 females) with a normal histologic examination of their sentinel lymph node were included. They belonged to a series of sixty-one melanoma patients undergoing lymph node excision. Fourteen complications were observed in ten patients. Two early complications were seen: hematoma (n=1) and deep venous thrombosis with pulmonary embolism (n=1). Twelve late complications were observed: mild lymphoedema (n=5), hypertrophic scars (n=2), painful scars (n=4), and one chronic seroma (n=1). Many complications (33%) were observed after excision in the inguinal area. DISCUSSION: The complications of sentinel lymph node excision must be considered in determining the benefit/risk ratio of this technique.

[Influence of the size and the localization of metastases in melanoma sentinel lymph nodes on the results of lymphadenectomy]. / Influence de la taille et de la localisation des métastases dans les ganglions sentinelles de mélanome sur le résultat du curage, à propos de 35 cas.

AIM: Thirty-five cases of lymphadenectomy carried out in the context of positive sentinel lymph node for malignant melanoma have been reviewed to assess the prognostic value of certain metastatic charachteristics. We have checked wether the type (macro or micrometastasis) and localisation (subcapsular or intraparenchymal) in the sentinel lymph node had predictive value for the lymphadenectomy outcome and evolution of the case. MATERIAL AND METHODS: The retrospective study relates to 35 cases (with an average 2 years history) taken from a total of 87 sentinel lymph node protocols; average age 46.5 years, Breslow 2.5 mm with an history of 25 months. RESULTS: Among the 35 positive sentinel lymph nodes we have 19 cases (54.2%) of micrometastasis. Among the 35 lymphadenectomy 5 cases (14.28%) turned out positive, 3 of which concerned micrometastatic sentinel lymph nodes. In our cohort the micrometastatic nature of sentinel lymph nodes did not have statistically significant impact upon the lymphadenectomy result but showed more favourable short-term evolution with 68.42% metastatic free evolution as against 43.75% in case of initial macrometastasis. The subcapsular localisation of micrometastasis equally represents a factor of improved prognosis (69.2% of metastatic free evolution against of 30.8% in the case of intraparenchymal localistion). CONCLUSION: Unfortunately, none of the studied criteria justifies a modification of our present clinical attitude whereby a systematic lymphadenectomy in cases of positive sentinel lymph nodes is performed, whatever the type or localisation of the relevant metastases.

[Cutaneous vasculitis with necrotic ulcers in rheumatoid arthritis: treatment with anti-TNFalpha]. / Vasculite avec ulcérations cutanées au cours de la polyarthrite rhumatoïde: traitement par anti TNFalpha.

INTRODUCTION: Anti-TNFalpha has occasionally been used in the treatment of recalcitrant forms of systemic vasculitis such as Behçet's disease, Wegener's granulomatosis and Churg-Strauss syndrome. We report on the outcome of treatment in rheumatoid arthritis patients with cutaneous vasculitis lesions on anti-TNFalpha. OBSERVATIONS: Two patients with rheumatoid arthritis present for several years had necrotic ulcers of the lower limbs due to cutaneous vasculitis. After the failure of various immunosuppressive drugs (cyclophosphamide, azathioprine, methotrexate), the two patients were treated with anti-TNFalpha: infliximab in the first case and adalimumab in the second. Cutaneous ulcers healed within two to four months of the start of anti-TNFalpha treatment. Despite ongoing anti-TNFalpha treatment, these cutaneous ulcers relapsed four to six months after complete healing. CONCLUSION: Initially spectacular healing of cutaneous vasculitis ulcers under anti-TNF alpha treatment followed by relapse after several months of treatment is suggestive of an escape mechanism.

[Cyclophosphamide-induced neutrophilic disease in a patient with angioimmunoblastic lymphadenopathy and myelodysplastic syndrome]. / Dermatose neutrophilique, au décours d'une lymphadénopathie angio-immunoblastique, avec dysmyélopoïèse induite par le cyclophosphamide.

BACKGROUND: Cutaneous involvement in patients with angioimmunoblastic lymphadenopathy is often poorly specific for maculopapular eruptions. We report an atypical case of neutrophilic disorder associating subcorneal pustules and hypodermal nodules in a male patient with angioimmunoblastic lymphadenopathy. CASE REPORT: A 62 year-old man with angioimmunoblastic lymphadenopathy was treated with oral prednisone (15 mg/day) and cyclophosphamide. Pustules and deep nodules occurred simultaneously on the arms and trunk, the abdomen and the back respectively. Biopsy of these lesions showed an infiltration of neutrophil polymorphonuclears in the median and superficial dermis with a subcorneal pustule and nodular infiltration of neutrophil polymorphonuclears in the deep dermis and hypodermis respectively. Increasing the dose of prednisone to 30 mg/day led to rapid and complete healing of the cutaneous lesions. DISCUSSION: This atypical case of neutrophilic disorder associating acute subcorneal pustules and deep dermal-hypodermal nodules seems to correspond to a unique type of neutrophilic disorder that differs from those previously reported in the literature. Neutrophilic disorders have been reported only rarely in patients with angioimmunoblastic lymphadenopathy. This case illustrates the wide range of clinical and histological lesions found in neutrophilic disorders, a number of which may coexist in a single patient.

[Dyspareunia and Sjögren's syndrome]. / Dyspareunie et syndrome de Sjögren.

BACKGROUND: Dyspareunia is frequently associated with a psychiatric origin, particularly in patients with no obvious vulvovaginal or pelvic disease. The aim of this study was to assess the frequency of dry syndrome in patients with dyspareunia and to evaluate the main clinical and biological features and follow-up data for women with dyspareunia and dry syndrome. PATIENTS AND METHODS: Twenty-two patients presenting chronic idiopathic dyspareunia (without clear vulvovaginal dermatosis or infection) were included in this retrospective study. All patients underwent history-taking, gynecological examination, a Schirmer tear test, a sugar test, labial salivary gland biopsy assessment and immunological examination. A diagnosis of Sjögren's syndrome was considered where histological examination of the salivary glands showed a lymphocyte infiltration corresponding to stage 3 or 4 in the Chisholm classification. Diagnosis of dry syndrome without Sjögren's syndrome was made in patients with xerostomia and/or xerophthalmia without a specific histological picture of Sjögren's syndrome or immunological abnormalities at salivary gland biopsy. RESULTS: Based on our criteria, 10 patients (45%) had dry syndrome, including 4 with Sjögren's syndrome and 6 with dry syndrome without Sjögren's syndrome. 9 of these 10 patients presented either xerostomia (7 cases) and/or xerophthalmia (7 cases). Vaginal dryness was reported by 3 of the 10 women with dry syndrome but also by 4 of 12 women without dry syndrome. Examination of the vulva showed no particular clinical features and treatment with an emollient was not effective in all cases. DISCUSSION: This study showed a high frequency of dry syndrome in patients with chronic "idiopathic" dyspareunia. The incidence of the condition was even greater in women with functional conditions evocative of dry syndrome. Women presenting dyspareunia with no clearly related clinical causes should thus be carefully assessed for dry syndrome.

[The importance of flow cytometry and the cell proliferation index in choroidal melanoma]. / Intérêt de la cytométrie en flux et de l'index de prolifération cellulaire dans le mélanome choroïdien.

PURPOSE: To evaluate the prognostic value of DNA ploidy and proliferative activity in metastasis occurrence after enucleation for choroidal melanomas. METHODS: This retrospective study investigated 14 patients (eight males, six females) using flow cytometry and immunohistochemistry with the Ki67 antibody. RESULTS: Two of the tumors were aneuploid, 12 were diploid. The proliferation index was between 0% and 16%. Two tumors progressed to metastatic disease; both were diploid and had a low proliferation index. They were mixed cell type. The median observation period was 3 years and 9 months. CONCLUSION: DNA content and proliferative activity are not correlated with metastasis occurrence. Sclera invasion is the best prognostic factor.