[Merkel cell carcinoma: a new radiation-induced cancer?]. / Carcinome de Merkel : un nouveau cancer radio-induit ?

BACKGROUND: Merkel cell carcinoma (MCC), a rare and aggressive neuroendocrine tumour, appears primarily on sun-exposed areas in light-skinned elderly subjects. UV exposure and profound immunosuppression (particularly in a setting of solid organ transplantation, haematological malignancies, HIV) constitute the principal risk factors. The aetiopathogenesis of this cancer is not known, although a polyomavirus involved in the oncogenic process was recently discovered. The carcinogenic effect of ionizing radiation, while not clearly established, has been suspected in rare cases involving the onset of MCC in irradiated zones. We report a new case of case of MCC in a patient previously undergoing radiotherapy. CASE REPORT: A 59-year-old-man underwent radiotherapy for a Darier-Ferrand dermatofibrosarcoma on the left shoulder and developed MCC at the same site 38 years later. DISCUSSION: The time between radiotherapy and diagnosis of MCC, its site within the radiation field (radio-dermatitis), the description of similar cases in the literature concerning the onset of MCC in irradiated areas, and the known carcinogenic effects of ionizing radiation all militate strongly in favour of the radiation-induced nature of MCC.

[Histologic risk factors of basal cell carcinoma of the face, about 184 cases]. / Facteurs de risques histologiques des carcinomes basocellulaires de la face : à propos d'une série de 184 cas.

BACKGROUND: Basal cell carcinoma is the most common type of skin cancer in humans. OBJECTIVES: The aim of our study was to determine the histologic risk factors involved in recurrence of basal cell carcinomas of the face. PATIENTS AND METHODS: We conducted a retrospective study of patients with primary basal cell carcinoma (BCC) of the face treated between March 2003 and December 2005. We analyzed the size of lateral and deep margins of tumor, histologic subtype, perineural invasion, and ulcerations. Clinical follow-up was observed until June 2011. RESULTS: We note that 184 cases of BCC were included. Eleven recurrences occurred during the follow-up, i.e. 6%. The population was divided into two groups according to histologic safety margins (1 mm for all basal cell carcinomas, 0.8 mm for nodular and 2 mm for aggressive-growth (AG-BCC) subtypes). There was a significant difference between groups in regards to cancer recurrence. Tumor size above 2 cm and presence of perineural invasion increased the risk of recurrence. DISCUSSION: Low histological safety margins appear to be critical on tumor recurrence. Depending on the tumor characteristics, and the patient, we advocate a re-excision in cases of histological safety margins inferior to 0.8 mm for the nodular subtypes and 2 mm for aggressive subtypes. Tumor size, and perineural invasion should be taken into consideration so as to make a well-informed decision between re-excision and a watching strategy in critical cases.

Clinicopathological study of 13 cases of squamous cell carcinoma complicating hidradenitis suppurativa.

BACKGROUND: To the best of our knowledge, only 52 cases of squamous cell carcinoma (SCC) complicating hidradenitis suppurativa (HS) have been reported since 1958. We describe 13 new cases. METHODS: We propose a clinical and histological analysis of our cases. We include these results in a review of previously reported cases to analyze a total of 65 patients. In our series of 13 cases, we also investigate the presence of human papillomavirus (HPV) in tumor samples, by polymerase chain reaction (PCR) on paraffin-embedded material. RESULTS: Malignant transformation affects mainly men with a long-term history of genitoanal HS. Although our cases were 7 well-differentiated carcinomas and 6 verrucous carcinomas, lymphatic and visceral metastasis occurred in 2 and 3 cases, respectively. With PCR, we demonstrated presence of HPV in genitoanal tumoral lesions, principally HPV-16. CONCLUSION: SCC complicating HS evolves poorly, despite a good histological prognosis. Our results sustain the implication of HPV in the malignant transformation of HS.

[Giant keratoacanthoma of the eyelid]. / Kératoacanthome géant de la paupière.

The clinicopathologic case of a 76-year-old male with a giant tumor of the left eyelid is reported. Histopathological diagnosis was a keratoacanthoma. The lesion was treated with an injection of 5-Fluoro-Uracil and oral treatment with acitretin (Soriatane), causing the lesion to disappear quickly. Clinical and histopathological characteristics of keratoacanthoma are important to know because the differential diagnosis is epidermoid carcinoma. This treatment is original in that the surgical exeresis usually done in this kind of tumor would have been too mutilating for the eyelid in such a case.

[Giant primitive neuroendocrine cutaneous carcinoma of the leg]. / Carcinome neuroendocrine cutané primitif géant de la jambe.

INTRODUCTION: Primitive cutaneous neuroendocrine carcinoma or Merkel cell carcinoma is a tumor characterized by rapid evolution. The size at the time of diagnosis is a mean of 2 to 3 centimeters. We report a case of a particular clinical and anatomopathological presentation. OBSERVATION: A 76 year-old woman had a voluminous tumoral plaque on the left leg with multiple ulcero-necrotic lesions involving the bone structure without palpable, inguinal lymphadenopathy. The histological and immunocytochemistry examination led to the diagnosis of cutaneous neuroendocrine carcinoma. The normality of the chest, abdominal and pelvis investigations confirmed the primitive cutaneous origin. The patient died a few months later without lymph involvement. DISCUSSION: To the best of our knowledge, such a presentation of primitive cutaneous neuroendocrine carcinoma has never been reported. The absence of lymphadenopathy despite the tumor volume and extensive skin involvement suggest the hypothesis of a non lymphophilic form of primitive cutaneous neuroendocrine carcinoma. This case report shows that lymphophily depends neither on the tumoral size nor to the degree of differentiation of tumoral cell proliferation, but on biological factors that require specification.

Juvenile xanthogranuloma: a congenital giant form leading to a wide atrophic sequela.

Juvenile xanthogranuloma is the most common non-Langerhans cell histiocytosis of infancy and childhood. It is a benign disorder that usually regresses spontaneously, more often without or with limited skin changes. The clinical features are very pleomorphic and recognition of the atypical presentations should facilitate the diagnosis. We report a 9-month-old boy who had an extensive, atrophic lesion on the proximal thigh that followed the course of the congenital giant form. The large size of the lesion does not alter the clinical course or result in extracutaneous involvement, which remains rare. However, uncommon clinical presentations raise the problem of differential diagnosis, requiring biopsy to eliminate other tumors with a less favorable prognosis.

Progression of actinic keratosis to squamous cell carcinoma of the skin correlates with deletion of the 9p21 region encoding the p16(INK4a) tumor suppressor.

Actinic keratoses (AKs) are pre-neoplastic lesions that can develop into squamous cell carcinomas (SCCs) of the skin. Often AK and SCC have commonly altered p53. A status of another tumor suppressor, the p16(INK4a), was reported for SCC but not for AK. A comparative study of SCC and AK human samples by loss of heterozygosity (LOH) analysis determined that the p16(INK4a/ARF) locus is less frequently altered in AKs than in SCCs. These LOH data highly correlated with immunohistochemical findings demonstrating the presence of p16(INK4a) in the AK skin samples but its absence in SCC lesions. Our results imply that progression of AK into SCC may involve inactivation of p16(INK4a).

Pancreatoblastoma in childhood: clinical course and therapeutic management of seven patients.

BACKGROUND: To analyze the clinical course of pancreatoblastoma in children and to propose management and therapy. PROCEDURE: Retrospective review of seven cases of pancreatoblastoma treated in France over a 20-year period and literature review. RESULTS: Median age of patients was 6 years. The tumor was well encapsulated in one patient and had direct extension to adjacent tissues in two others. Three patients had regional lymph node involvement and one had liver metastases. The tumor arose in the head of the pancreas in three children, in the tail and body in two and in the tail and in the body, one each. Elevation of AFP serum levels was noted in four out of 6 cases. Five tumor resections were performed, one initially and 4 after neoadjuvant chemotherapy, and cisplatin plus doxorubicin seemed effective. Two children received post-operative irradiation because of incomplete resection. In all, four children are disease free with a median follow-up of 50 months ( range, 5--120 months) : one had a complete removal of tumor at diagnosis and no further treatment, three had unresectable tumor at diagnosis and received neoadjuvant chemotherapy with cisplatin and doxorubicin. One of them also received post-operative irradiation. CONCLUSIONS: Pancreatoblastoma is a curable tumor. Examination of serum AFP levels may be useful for diagnosis and to follow the course of the disease. Complete resection is the treatment of choice. However, tumor is often unresectable at diagnosis and preoperative chemotherapy is needed to reduce tumor volume. We suggest a regimen that include cisplatin and doxorubicin. In patients with incompletely resected disease, postoperative radiation may be indicated.

[Granuloma annulare following BCG vaccination]. / Granulome annulaire après vaccination par le BCG.

BACKGROUND: Granuloma annulare following BCG vaccination or tuberculin skin tests is rarely reported in the literature. We describe three cases occurring in patients under the age of 6 years. CASE REPORTS: In the first two cases, granuloma annulare was initially localized at the vaccinal site, then generalized. In the third case, diagnosis was deep granuloma annulare localized far from the initial vaccination site, with recurrence following tuberculin test. In the three cases, diagnosis was made on the basis of clinical and histological elements. DISCUSSION: The relationship between granuloma annulare and BCG may seem evident when the eruption occurs at the vaccinal site, but remains difficult to prove. Diagnosis essentially relies on the absence of other classical etiologies and a compatible delay. When a vaccination such as BCG appears to be the cause of granuloma annulare, a possible cause would be injection trauma or a cell-mediated-delayed-hypersensitivity reaction to a specific antigen contained in the vaccine, leading to development of skin disorders such as granuloma annulare in predisposed subjects. Reports of granuloma annulare following BCG are uncommon but the incidence may be underestimated. In practice, diagnosis of granuloma annulare following BCG or tuberculin skin test cannot be established until BCG dissemination infection is ruled out, a necessary clinical and paraclinical step required in all cases of eruptions following this vaccination. Granuloma annulare is a benign complication requiring no treatment and does not constitute a contraindication for other usual vaccinations.

Cutaneous granulocytic sarcoma arising at the site of radiotherapy for breast carcinoma.

Carcinogenic effects of radiotherapy in breast cancer are well-known. Long-term follow-up of these patients shows a significantly increased risk of leukemia. Cutaneous granulocytic sarcoma is an uncommon leukemia cutis that usually occurs in association with acute myelocytic leukemia or myeloproliferative disorders. We report a case of cutaneous granulocytic sarcoma in a 44-year-old woman who had been treated six months earlier for breast adenocarcinoma. The treatment had associated lumpectomy, axillary lymph node dissection and radiotherapy. Skin lesions appeared firstly and predominantly on the irradiated area. Haematological investigations were normal and the diagnosis of isolated sarcoma was made. The uncommon features of this case were the short interval between radiotherapy and the occurrence of leukemia skin lesions and the fact that, to our knowledge, this is the first report of leukemia cutis localised on the irradiated area. The responsibility of radiation in the distribution of cutaneous lesions of granulocytic sarcoma is discussed.

[Rhabdomyosarcoma at site of pacemaker implantation]. / Rhabdomyosarcome sur site d'implantation d'un stimulateur cardiaque.

BACKGROUND: Malignant proliferation at the site of implantation of a pacemaker generator is uncommon. We report the case of a patient who developed rhabdomyosarcoma. CASE REPORT: A 85-year-old man presented with a voluminous and rapidly evolving tumor localised beneath the right clavicle. This inflammatory and necrotic lesion developed on the area where a titanium pacemaker had been implanted five years earlier. Rhabdomyosarcoma was diagnosis on the basis of immunohistochemistry findings. In spite of a wide surgical excision of this primitive tumour, visceral dissemination appeared, rapidly leading to the patient's death. DISCUSSION: This observation rises the question of the role of the pacemaker implantation in tumor development. The excellent in vivo tolerance and the widespread utilization of titanium as biomaterial is an argument against its carcinogenic action. Inversely, a metal-related chronic inflammatory reaction could favor the neoplastic process in predisposed subjects as has been observed with prosthetic materials used in orthopaedics.

[A case study of Woringer-Kolopp disease. Immunohistochemical study]. / Cas anatomoclinique de maladie de Woringer et Kolopp. Intérêt de l'étude immunohistochimique.

Woringer-Kolopp disease is a localized epidermotropic T cell lymphoma with good prognosis. We describe a 79-year-old man with an erythematous scaly plaque of the foot. Clinical diagnosis of psoriasis, parapsoriasis and fungal infection were proposed. Histopathological and immunohistochemical findings were characteristic of Woringer-Kolopp disease. We observed an immunohistochemical positivity of tumour cells for the anti-CD103 antibody (alphaEB7 integrin) according to the epidermotropism of this localized cutaneous T cell lymphoma.

[Uterine leiomyosarcomas: a surprising pathology. Review of the literature. Six case reports]. / Léiomyosarcomes utérins: une surprise anatomopathologique. Revue de la littérture. A propos de 6 observations.

OBJECTIVE: The aim of this study was to determine the clinical features and laboratory tests which define a population with a high risk of leiomyosarcoma. METHOD: A retrospective analysis of 660 hysterectomies performed between 1989 and 1994 was conducted. There were 414 cases of leiomyoma and 6 leiomyosarcomas (1.4%). RESULTS: The indication for hysterectomy was complication of fibromyomatous uterus in all cases. Diagnosis of leiomyosarcoma was never suspected before surgery and the pathology examination. Complimentary radiotherapy was given in 2 patients among the 6 with leiomyosarcoma. Mean survival was 35 months. There were no cases of recurrence or metastasis. None of the laboratory tests performed provided clues to diagnosis. CONCLUSION: Physicians should be aware of possible misdiagnosis of a leiomyosarcoma when proposing medical treatment of uterine fibroma.

[Bronchiolitis obliterans, pneumocystosis and HIV infection]. / Bronchiolite oblitérante, pneumocystose et infection par le V.I.H.

A 39 year old man who was HIV positive and was treated with trimethoprim-sulfamethoxazole for pneumocystis with hypoxaemia. During the acute episode he had a persistent fever of 38 degrees and hypoxaemia with a PaO2 of 65 mm/Hg and bilateral opacities both radiologically and on a CT scan, which were of alveolar type, with bronchograms identical to those observed before the treatment of the pneumocystis. In view of the negative evidence for a respiratory or extra respiratory infection, a surgical biopsy was performed and this revealed lesions of bronchiolitis obliterans with an organising pneumonia (BOOP). After the thoracotomy, there was a spontaneous clinical cure in a few days and radiological clearance in a month. This very rare diagnosis should be added to the list of causes of alveolar pneumopathy with infiltration and fever occurring during the course of an HIV infection.

[Popliteal venous aneurysm revealed by recurring pulmonary embolism. Echographic, phlebographic, x-ray computed tomographic and nuclear magnetic resonance aspects]. / Anévrisme veineux poplité révélé par des embolies pulmonaires récidivantes. Aspect échographique, phlébographique, tomodensitométrique et en résonance magnétique nucléaire.

We report a characteristic case of popliteal vein aneurysm which was demonstrated not only by Doppler ultrasonography and venographic examination, but also by CT scan and magnetic resonance imaging. A review of the literature underlines the rarity of these aneurysms, since less than 20 cases have been published. They are always true aneurysms, most often revealed after an episode of pulmonary embolism. Doppler ultrasonography and venography confirm the diagnosis. The place of CT scan and magnetic resonance imaging remains to be defined. Even if asymptomatic, the embolic risk necessitates surgical resection of the aneurysm and restoration of venous continuity.