Accessory cavitated uterine mass: MRI features and surgical correlations of a rare but under-recognised entity.

OBJECTIVES: To describe MRI features of accessory cavitated uterine mass (ACUM) with surgical correlations. METHODS: Eleven young women with an ACUM at pathology underwent preoperative pelvic MRI. Two experienced radiologists retrospectively analysed MR images in consensus to determine the lesion location within the uterus, its size, morphology (shape and boundaries), and structure reporting the signal and enhancement of its different parts compared to myometrium. The presence of an associated urogenital malformation or other gynaecological anomaly was reported. MRI features were correlated with surgical findings. RESULTS: All 11 lesions were well correlated with surgical findings, lateralised (seven were left-sided), and located under the horn and the round ligament insertion. Nine were located within the external myometrium, bulging into the broad ligament. Two were extrauterine, entirely located within the broad ligament. On MRI, the mean size was 28 mm (range 17-60 mm). Nine lesions were round-shaped, two were oval; all had regular boundaries. At surgery, the ACUM were not encapsulated but were possible to enucleate. On MRI, all lesions were well defined and showed a central haemorrhagic cavity surrounded by a regular ring (mean thickness, 5 mm) which had the same signal compared to the junctional zone. ACUM was isolated in all women, without urogenital malformation, adenomyosis or deep endometriosis. CONCLUSIONS: On MRI, ACUM was an isolated round accessory cavitated functional non-communicating horn-like aspect in an otherwise normal uterus. MRI may facilitate timely diagnosis and appropriate curative fertility-sparing laparoscopic resection. KEY POINTS: • ACUM is rare, with delayed diagnosis in young women with severe dysmenorrhoea. Pelvic MRI facilitates timely diagnosis and appropriate curative fertility-sparing laparoscopic resection. • Quasi-systematically located under the uterine round ligament insertion, ACUM may be intramyometrial and/or in the broad ligament. • On MRI ACUM resemble a non-communicating functional accessory horn within a normal uterus; the mass, most often round-shaped, had a central haemorrhagic cavity surrounded by a regular ring which had the same low signal compared to the uterine junctional zone.

[A new case of systemic contact eczema to nickel in a peripheral venous catheter]. / Eczéma de contact systémique au nickel d'un cathéter périphérique : un nouveau cas.

BACKGROUND: Nickel-elicited systemic contact dermatitis is a rare event seen in previously skin sensitized patients. We report a case of systemic contact dermatitis due to nickel released into the bloodstream from a metal section of a catheter during infusion. CASE REPORT: A 39-year-old woman presented papular and vesicular flexural dermatitis and pompholyx 72h after cervical spine surgery. She received numerous treatments during the perioperative period. A challenge test with one of the suspected treatments, cefazolin, was performed by intravenous infusion over a six-hour period using the same Optiva) peripheral catheter (Johnson & Johnson, USA). Six hours after withdrawal of the catheter, an eruption occurred. A further cefazolin challenge test performed later under identical conditions but using a different type of catheter (nickel-free) was negative. Nickel-elicited systemic contact dermatitis due to nickel release from a catheter was diagnosed. The patient's medical history was notable for contact dermatitis with jewellery. Patch tests confirmed marked nickel sensitization. DISCUSSION: A little-known way of systemic nickel absorption is through insertion of a venous catheter with a metal section containing nickel and a metallic eyelet containing nickel can in fact remain in place after catheter placement. Nickel can thus be released into the circulation during infusion and an eruption may occur during the postoperative period. This diagnosis is noteworthy as such eruptions can easily be mistakenly diagnosed as cutaneous drug eruptions.

Nickel-elicited systemic contact dermatitis from a peripheral intravenous catheter.

Nickel-elicited systemic contact dermatitis is a well-known entity, although it is far less common than allergic contact dermatitis. In most of the cases, the main way of nickel administration is oral. Clinical manifestations are miscellaneous including pompholyx, diffuse exanthema, flexural dermatitis or baboon syndrome. Systemic nickel dermatitis induced by venous catheters is very uncommon, but it is probably underdiagnosed. We report here 2 patients with diffuse recurrent maculopapular rash corresponding to nickel-elicited systemic contact dermatitis. They were both perfused during the last episode with the assistance of a peripheral polyurethane venous catheter during or just before the cutaneous eruption. At the base of the catheter, there was a small metallic eyelet on which dimethylglyoxime test was positive, indicating a release of nickel. Then, we measured nickel release in normal use conditions and found high nickel levels, although the manufacturer denied that nickel could be released. This diagnosis is important to know because such exanthema often occurred during postoperative or postpartum period. Its frequency is probably underestimated because it is often considered as a cutaneous drug reaction. To our knowledge, only 2 cases have been reported in the literature.

Delays in diagnosis and melanoma prognosis (I): the role of patients.

A prospective survey was conducted to assess the role of patients in the melanoma prognosis. Consecutive patients with primary melanoma were interviewed and examined using a comprehensive questionnaire including a psychological instrument. Main outcome measures were the delay before medical intervention and the tumor thickness. Of 590 melanomas, 70.8% were detected by patients and this proportion was higher in females. Relatives were involved in the detection of half of the cases. Median delays before the patient realized he had a suspicious lesion, before this lesion was seen by a doctor, and before the melanoma was removed were 4 months, 2 months, and 1 week, respectively. Delays up to several years were observed in some cases. The rate of self-detection tended to be lower, the delays before seeking medical advice to be longer, and the tumor thickness to be higher in old people, in males, in lower-educated individuals, in those living out of towns, and in people with a low awareness about melanocytic tumors than in other cases. Conversely, individuals with a high number of atypical nevi, those who were aware to be at risk, and those who regularly visited a dermatologist tended to detect their melanoma more rapidly. No specific psychological traits were associated with a late reaction, although negligence and anxiety tended to prolong the delays. Knowledge about melanoma was poor in many patients, especially in males, and wrong beliefs were widespread. This study provides the targets of future education programs.

Delays in diagnosis and melanoma prognosis (II): the role of doctors.

A prospective survey was conducted to assess physician responsibility in melanoma prognosis. Consecutive patients with primary melanoma were interviewed and examined using a standardized questionnaire. Main outcome measures were medical components of the delay before tumor resection and tumor thickness. Of 590 melanomas, 29.1% were coincidentally detected by physicians and their tumor depth was lower than in melanomas detected by patients (p < 0.001). Physician sensitivity for melanoma diagnosis was evaluated at 86%. Median time intervals to propose resection and to perform removal of melanoma were short: 0 (mean 103) and 7 (mean 68) days, respectively. Melanomas were managed in an inappropriate way in 14.2% of cases. Location on acral areas and absence of pigmentation were associated with longer medical delays and more frequent inappropriate medical attitudes. Melanomas located on hardly visible areas were less frequently detected by physicians than those on visible areas. Medical delays were shorter, doctor's attitude was more frequently appropriate, and melanoma thickness was lower (p < 0.001) when the patient visited a dermatologist (54.7%) than when he or she visited a general practitioner (33.4%). Our study shows that doctor responsibility accounts for only a small part of the total delay before melanoma removal. However, systematic total examination and better training of doctors, especially about unusual forms of melanoma, could still improve melanoma detection.

Angiosarcoma of the scalp and face: failure of an interferon alpha treatment.

Angiosarcoma of the scalp and face is a rare malignant endothelial tumor arising in elderly people. Treatment is disappointing and prognosis remains poor. We report two cases of angiosarcoma of the scalp and face relapsing after classical therapy with surgery and radiotherapy and treated with interferon alpha. The tolerance was poor and the disease progressed 2 and 5 months after the onset of the treatment. When used alone, interferon alpha does not appear as an effective treatment in this kind of angiosarcoma.

[Extensive primary cutaneous mucinosis associated with refractory anemia with excess of blast]. / Mucinose cutanée primitive diffuse associée à une anémie réfractaire avec excès de blastes.

BACKGROUND: We describe an original case of a primary cutaneous mucinosis, associated with a refractory anemia with excess of blast. CASE REPORT: A 65 year-old female consulted for erythematous-purple blush reticulated and large plaques that progressively developed on the trunk and limbs. Biopsies showed abundant dermis mucin deposits with a perivascular lymphocytic infiltrate. The epidermis was normal. Laboratory evaluation showed refractory anemia with excess of blast, associated with cytogenetic abnormalities. After two years course, the progressive aggravation of the myelodysplastic syndrome was associated with a progressive extension of the cutaneous mucinosis. DISCUSSION: This is the first report of an atypical primary diffuse and extensive cutaneous mucinosis. Primary cutaneous mucinosis may be associated with systemic diseases. To our knowledge, the association with refractory anemia with excess of blast has not been described previously. Mucine deposits may be considered as a consequence of neutrophilic dysfunction that are observed in myelodysplastic syndromes.

Notalgia paresthetica: clinical, physiopathological and therapeutic aspects. A study of 12 cases.

Notalgia paresthetica (NP) is a common but often unrecognized neurocutaneous condition, with very few cases reported to date. It is characterized by pruritus localized in an area between D2 and D6 dermatomes, sometimes accompanied by sensory neuropathies and/or electrical conductivity disorders. Cutaneous pigmented patches and friction amyloidosis can arise with irritation. Some hereditary cases have been noted mainly in young patients, associated with multiple endocrine neoplasia type 2A. However, NP mainly occurs in older patients and most are sporadic pathologies linked with musculoskeletal compression of spinal nerves. Only capsaicin has shown some (but unfortunately only transient) efficacy in relieving NP symptoms. We present observations on 12 sporadic cases of NP. Spinal X-rays revealed dorsal arthrosis or spinal static disequilibrium in nine of these patients. Six patients underwent spinal and paraspinal ultrasound or radiation (better) physiotherapy, and the symptoms subsided in four of these cases. These results highlighted that spinal disorders could be a determining factor in NP, indicating that patients could benefit from physiotherapy.

Melanoma and tumor thickness: challenges of early diagnosis.

OBJECTIVE: To test the basic assumption of campaigns for early diagnosis of melanoma, ie, prognosis is correlated with the delay in the diagnosis. DESIGN: Prospective study of the correlation between delays to diagnosis, assessed using a questionnaire, and the Breslow thickness as a prognosis marker. SETTING: Dermatology departments in France. PATIENTS: Five hundred ninety consecutive patients, referred within 12 weeks after resection of cutaneous melanoma. MAIN OUTCOME MEASURES: Assessment of 5 successive time intervals from the first time the patients realized that they had a lesion until the resection of the melanoma, and results of the correlation between each time interval and tumor thickness (Breslow). RESULTS: There is a positive but weak correlation between tumor thickness and the delay to identify a lesion as suspicious (r = 0.17; P = .009). However, this delay tends to be short for the thickest tumors. There is a negative correlation between tumor thickness and the delay to seek medical attention (r = -0.20; P<.001). This delay was shorter for nodular melanoma. No correlation is found between melanoma thickness and physicians' delays. CONCLUSIONS: Poor prognosis can be accounted for by aggressive rapidly growing tumors rather than by delays. In well-informed populations, campaigns for early diagnosis of melanoma may thus no longer have a major impact on prognosis, unless they are focused on subgroups less accessible to information and medical care.

[UV-induced immunosuppression and skin cancers]. / Immunosuppression photo-induite et cancers cutanés.

INTRODUCTION: The increased incidence of skin cancers is due to modifications of our behavior toward solar exposure. Photocarcinogenesis represents the sum of complex and intricate events that lead to the occurrence of skin cancers. CURRENT KNOWLEDGE AND KEY POINTS: In epidermal cells UV light induces lesions of DNA that lead to modifications in oncogene and tumor suppressor gene expression. UV-induced immunosuppression is also important for tumoral promotion. UV exposure decreases the number of Langerhans cells in the epidermis and modifies their antigen-presenting cell capacity. Numerous experimental data obtained in animal models clearly indicate the existence of a relationship between UV-induced immune suppression and skin cancers. In humans, growing evidence suggests that skin cancers and photoimmunosuppression are linked. FUTURE PROSPECTS AND PROJECTS: Better knowledge of mechanisms involved in UV-induced immune suppression is essential for developing new strategies aimed at photoprotection and cancer prevention.

[Malignant tumors as complications of lymphedema]. / Les complications tumorales malignes des lymphoedèmes.

Malignant tumour arising in chronic congenital or acquired lymphedema is a rare medical condition. However it must be well known because of the usual severity of these highly malignant neoplasms. Stewart-Treves angiosarcoma is the most frequent tumour: it is a vascular malignant tumour, which mostly occur in post-surgical or radiotherapeutic lymphedema for breast cancer. It differs from secondary metastasis arising in lymphedema although the clinical and histological appearance is often very close. Other tumours can grow on this ground: Kaposi's sarcoma, squamous cell carcinoma, malignant lymphoma, melanoma. On the pathogenic point of view, many factors converge to this tumoral occurrence: lymphatic stasis and the reorganization of the conjunctival tissue that it will entail, the local immunodeficiency, and also systemic factors as a general immunodeficiency or viral potentially oncogenic infections such as papillomavirus. In practice, the prevention of lymphedema, the regular monitoring of constituted lymphedemas, the hygienic and preventive behaviours of infections are the best arrangements to avoid this tumoral occurrence.

[Phakomatosis pigmento-vascularis. Report of 2 cases associated with angiodysplasia]. / La phacomatose pigmento-vasculaire. A propos de 2 cas associés à une angiodysplasie.

Phakomatosis pigmentovascularis is an uncommon disease, with a peculiar association of capillary hemangioma and pigmented lesions. Four entities have to date been described (I to IV), with localized (a) or systematical (b) involvement. In this latter subtype, the cutaneous lesions are associated with visceral (eye, central nervous system) and bony abnormalities. We describe two additional cases of phakomatosis pigmentovascularis type II (b) associated with a Klippel-Trenaunay syndrome. These reports emphasize the frequent occurrence of angiodysplasia of the Klippel-Trenaunay or Sturge-Weber-Krabbe type in the systemic subtype, especially II (b). Accordingly, complete investigations are warranted in all cases, with special attention for bones and some internal organs like eye and central nervous system. Pathophysiological hypothesis for phakomatosis pigmentovascularis are discussed.