[Immunoglobulin G4-related lung disease: Case report and literature review]. / Maladie à immunoglobuline G4 pulmonaire : rapport de cas et revue de littérature.

We report the case of a 61-year-old man with a pulmonary nodule discovered while staging the metastasis of a squamous cell carcinoma. No primary cancer was found. Histological examination of the resected specimen (lingula) was in favor of IgG4-related inflammatory pseudotumor. Histological criteria were described in 2012, combining a dense lymphoplasmacitic infiltrate rich in IgG4-positive plasma cell, storiform fibrosis and obliterative phlebitis. IgG4/IgG plama cell ratio>40 % is mandatory for histological diagnosis of IgG4-related disease. This is a rare and often underdiagnosed disease. Diagnostic criteria are now defined and consensual, combining clinical signs, biology and histology.

The "spaghetti technique": an alternative to Mohs surgery or staged surgery for problematic lentiginous melanoma (lentigo maligna and acral lentiginous melanoma).

BACKGROUND: Lentigo maligna (LM) and acral lentiginous melanoma (ALM) are often large and clinically ill defined. The surgical challenge is to spare tissue while still achieving clear margins. OBJECTIVE: We sought to provide a retrospective assessment of a two-phase surgical technique for lentiginous melanomas (MM) not suitable for en bloc resection. METHODS: In the first phase, a narrow band of skin, "the spaghetti", is resected just beyond the clinical outline of the MM, immediately sutured, and sent for pathological examination without removing the MM. The same procedure is repeated beyond the segments which are shown to be not tumor free and so forth until the minimal tumor-free perimeter is outlined. No operative wound is left between operative sessions. In the second phase, the MM resection and reconstruction are performed at the same time. RESULTS: In 21 patients with LM (n = 16) or ALM (n = 5), the mean operative defect size was 27.5 cm(2) (range, 1.97-108.4 cm(2)). The mean number of steps in the procedure was 1.55 (1-4). Grafts were used for reconstruction in all cases. The relevance of the "spaghetti"-defined outline was confirmed in 19 of 21 patients. After a median follow-up period of 25.36 months (range, 0-72 months), the local control rate was 95.24% with one case (4.76%) of in-transit invasive recurrence after 48 months. LIMITATIONS: This study was performed at a single center and included a limited number of patients. The follow-up time was relatively brief. CONCLUSION: The "spaghetti technique" is simple and reliable for LM and ALM. Unlike Mohs surgery, it does not require specific training of surgeons or pathologists. Unlike staged surgery, it does not leave patients with an open wound on the face or soles before final reconstruction.

[Placental metastasis of melanoma: a new case and literature review]. / Métastase placentaire de mélanome : un nouveau cas et revue de la littérature.

We report a case of placental metastasis of melanoma in a 30-year-old woman, without fetal involving, the child being healthy after 5 months of follow-up. Placental or fetal metastasis of maternal cancer are rare, but melanoma is remarkable by its metastatic potential among the cancers of the woman in age to procreate. Thirty cases of placental or foetal metastasis of melanoma have been reported. The fetal involvement seems to be always associated with at least a microscopic invasion of the placenta, that is why systematic microscopic examination of the placenta in case of maternal cancer needs to be encouraged. The other predictive factors of fetal involvement seem multiple and complex. The follow-up of the children arisen from a mother affected by a cancer must be close even if, in case of metastasis in children, the prognosis is very poor.

[Alveolar microlithiasis with severe interstitial fibrosis leading to lung transplantation]. / Microlithiase alvéolaire avec fibrose interstitielle sévère conduisant à la greffe.

Pulmonary alveolar microlithiasis is a rare disease, characterized by extensive phosphocalcic concretions within the alveolar spaces. Pulmonary alveolar microlithiasis is usually asymptomatic and is incidentally found because radiologic findings are characteristic. In about half of the cases, it is an autosomal recessive disorder due to mutations in the SLC34A2 gene. Pulmonary alveolar microlithiasis can easily be diagnosed by bronchioloalveolar lavage or transbronchial biopsy. The clinical course is usually stable during several years and lung transplantation is the only effective treatment when a respiratory failure occurs. A 49-year-old woman was referred with a restrictive respiratory failure due to a pulmonary alveolar microlithiasis incidentally discovered on a chest radiography when she was 11 and was confirmed by surgical lung biopsy. She was asymptomatic until she was 43 when she presented a progressive dyspnea leading to continuous oxygen administration 4 years later. Laboratory findings only showed a polyglobulia related to hypoxemia. Chest radiography and computed tomography chest scan revealed a bilateral symmetric micronodular pattern. She underwent a lung transplantation when she was 49. Pathological examination confirmed the diagnosis of diffuse pulmonary alveolar microlithiasis with interstitial fibrosis. The patient died 3 months after surgery in an infectious context.

Unsuccessful rechallenge with pemetrexed after a previous radiation recall dermatitis.

Radiation recall dermatitis is characterized by an inflammatory reaction within a previously irradiated volume after administration of a drug. Antineoplastic drugs have mainly been involved in radiation recall reactions. This phenomenon is well known but poorly understood. Many hypotheses as stem-cell depletion in the radiotherapy field, heritable mutations within surviving stem cells, local vascular changes as well as a drug hypersensitivity reaction have been proposed to explain these reactions. In this report, we describe a non-small cell lung cancer patient treated with a carboplatin plus gemcitabine combination chemotherapy as first line followed by pemetrexed as second line therapy. Twenty-five years ago, she completed radiation therapy for breast cancer. Three days after the first cycle of pemetrexed, she presented with a radiation recall dermatitis. As EGFR-staining was negative, we rechallenged the patient with pemetrexed. Unfortunately, although less intense, we faced a recurrence of the skin reaction and pemetrexed was no longer continued.

From pancreatic intraepithelial neoplasia to cancer: a dramatic progression with K-ras status analysis.

Pancreatic ductal carcinomas are thought to arise from precursor ductal lesions called pancreatic intra-epithelial neoplasias or PanINs. We report the case of a woman suffering from idiopathic chronic pancreatitis associated with PanINs lesions who developed six years later an invasive ductal carcinoma. Immunohistochemistry for p53, HER-2/neu and genetic analysis of K-ras oncogene were performed at different stages of disease and revealed that the PanINs and the carcinoma did not express p53 and HER-2/neu gene products whereas a K-ras mutation was present at the carcinoma stage. The relationship between cancer and chronic pancreatitis and the main difficulties concerning the early diagnostic of pancreatic cancer are discussed.

Vancomycin-induced Henoch-Schönlein purpura: a case report.

INTRODUCTION: Henoch-Schönlein purpura is a small-vessel systemic vasculitis. Although its exact pathophysiology remains unknown, Henoch-Schönlein purpura has been reported in association with various medical conditions including hypersensitivity. We report the case of a patient with vancomycin-induced Henoch-Schönlein purpura. CASE PRESENTATION: A 42-year-old Caucasian man who had previously undergone a heart transplant was diagnosed as having an intra-abdominal abscess after he underwent a Hartmann procedure. At 15 days after initiation of antibiotic therapy including vancomycin, he developed a purpuric rash of the lower limbs, arthralgia, and macroscopic hematuria. At that time, our patient was already on hemodialysis for end-stage renal disease. Henoch-Schönlein purpura was diagnosed. After a second 15-day course of vancomycin, a second flare of Henoch-Schönlein purpura occurred. Skin biopsies showed leucocytoclastic vasculitis with IgA deposits and eosinophils in the peri-capillary inflammatory infiltrate, suggesting an allergic mechanism. After vancomycin was stopped, we did not observe any further flares. Only five cases of isolated cutaneous vasculitis, one case of lupus-like syndrome and one case of Henoch-Schönlein purpura after vancomycin treatment have been described to date in the literature. CONCLUSIONS: Clinicians should be aware that systemic vasculitis can be induced by some treatments. Vancomycin is a widely prescribed antibiotic. Occurrence of rare but serious Henoch-Schönlein purpura associated with vancomycin requires its prompt discontinuation.

Coffin-Siris syndrome with multiple congenital malformations and intrauterine death: towards a better delineation of the severe end of the spectrum.

Coffine-Siris syndrome or "fifth digit" syndrome is a multiple congenital anomaly-mental retardation syndrome with severe developmental delay, coarse facial features, hirsutism and absent fifth fingernails or toenails or fifth distal phalanges. The etiology of this syndrome remains uncertain. Here we report a stillborn male baby born from consanguineous parents who might represent a very severe form of Coffine-Siris syndrome with cardiac defect and multiple brain malformations including corpus callosum agenesis and Dandy Walker malformation. To the best of our knowledge, it is the first case leading to intrauterine death. Karyotype and array comparative genomic hybridization were normal; these results give additional support to mendelian inheritance for this syndrome. In our family, the most likely mode of inheritance is autosomal recessive and the recurrence is probably as high as 25%.

Extracapsular lymph node involvement is a negative prognostic factor after neoadjuvant chemoradiotherapy in locally advanced esophageal cancer.

INTRODUCTION: To assess prognosis depending on whether lymph node involvement (LNI) is intracapsular or with extracapsular breakthrough in patients with a locally advanced esophageal cancer treated with neoadjuvant chemoradiation and surgery. METHODS: Ninety-four consecutive patients with an esophageal cancer staged IIB (n = 17) and III (n = 77) received neoadjuvant chemoradiation followed by transthoracic esophagectomy with two-field lymphadenectomy. Histology was squamous cell carcinoma (n = 46) and adenocarcinoma (n = 48). Neoadjuvant therapy consisted of association of 5-fluorouracil/cisplatin concomitantly with a 45-Gy radiation therapy. Disease-free survival (DFS) excluding the in-hospital mortality was analyzed according to the nodal status and the invaded/resected lymph node ratio (LNR). Clinical factors affecting survival or predictors of extracapsular invasion were investigated by multivariate analysis. RESULTS: Five-year DFS rates were 46, 36, and 11% in N0 patients (n = 56), intracapsular LNI patients (n = 18), and extracapsular LNI patients (n = 10), respectively (p = 0.002). Intracapsular LNI patients with an LNR <0.1 (n = 12) had a 5-year DFS rate similar to N0 patients (44 versus 46%, p = 0.95). Intracapsular LNI patients with an LNR > or =0.1 (n = 6) had a DFS rate similar to extracapsular LNI patients (18 versus 11%, p = 0.69). Multivariate analysis revealed that the sole independent factor affecting DFS was the extracapsular LNI (HR = 3.9, p = 0.026). The number of invaded LN seemed to be the sole significant predictive factor for the development of ECLNI (HR = 2.39, p = 0.008). CONCLUSION: After neoadjuvant chemoradiotherapy, there was a significant difference on DFS depending on whether LNI was intracapsular or extracapsular. Extracapsular invasion seems to be an independent negative prognostic factor affecting survival, and its presence is related to the number of invaded LN.

Effect of hypertonic saline pre-treatment on ischemia-reperfusion lung injury in pig.

BACKGROUND: Hypertonic saline may be administered in the setting of lung transplantation but may affect the development of ischemia-reperfusion lung injury. This study investigated the effects of the pre-treatment by intravenous hypertonic saline in a pig model of single lung ischemia-reperfusion. METHODS: Forty-three pigs (34 +/- 4 kg) under mechanical ventilation were randomly assigned to a left lung ischemia-reperfusion alone or preceded by 4-ml/kg 7.5% hypertonic saline, 33-ml/kg normal saline, or by the infusion of the vasodilator nicardipine. Animals without ischemia served as controls. After euthanasia, the left lung was sampled for histologic analysis and measurement of lung water and alveolar-capillary permeability. RESULTS: Ischemia-reperfusion resulted in high-permeability pulmonary edema, hypoxemia, and increased interleukin-6 serum level. Hypertonic saline pre-treatment worsened pulmonary edema of the left lung (6.6 +/- 0.7 vs 4.8 +/- 0.8 ml/kg of body weight, p < 0.05) and resulted in a higher ratio of the protein level in the alveolar fluid to the serum protein level (0.41 +/- 0.04 vs 0.21 +/- 0.09, p < 0.05) and in a higher histologic damage score (11 [range, 9-11.75] vs 6.5 [range, 4.5-7.5], p < 0.05) without promoting pulmonary or systemic inflammation. Lung injury was affected neither by normal saline nor by nicardipine pre-treatment. Nicardipine did not influence the deleterious effect of hypertonic saline. CONCLUSIONS: Pre-treatment by intravenous hypertonic saline worsened ischemia-reperfusion lung injury independently of its effects on the cardiac index or pulmonary circulation but probably through a direct effect of hyperosmolarity on endothelial permeability.

Synchronous multiple primary lung cancer: an increasing clinical occurrence requiring multidisciplinary management.

OBJECTIVE: No guidelines detailing recommendations for the selection and treatment of patients with synchronous multiple primary lung cancer have been published. We report on a single-institution experience with synchronous multiple primary lung cancer, with emphasis on long-term survival. METHODS: We performed a retrospective study of 125 consecutive patients with synchronous multiple primary lung cancer who underwent operation between 1985 and 2006. Various treatment strategies were applied, including perioperative therapy. Potential prognosticators were submitted to univariate and multivariate analyses. RESULTS: Tumors were bilateral (n = 34) or ipsilateral (n = 91). Optimal surgical treatment (complete anatomic resection with radical lymphadenectomy) was possible in 65.6% of the cases. pN0 disease was present in 32.3% of the patients; 30-day and 90-day mortality rates were 4.5% and 11%, respectively. Two- and 5-year overall survivals were 61.6% and 34%, respectively, with a median survival of 35 months. On univariate analysis, smoking status, high Charlson index, low forced expiratory volume in 1 second, occurrence of postoperative complications, and performance of a pneumonectomy affected the overall survival adversely. Conversely, bilateral disease, location in the same lobe, and pN0 disease were favorable prognosticators. On multivariate analysis, low forced expiratory volume in 1 second, nonoptimal surgical treatment, and performance of a pneumonectomy were independent predictors of poor long-term survival, whereas female sex, younger age, asymptomatic disease, pN0 status, and performance of an adjuvant treatment affected the survival favorably. CONCLUSIONS: Provided there is an appropriate selection process, patients with synchronous multiple primary lung cancer are expected to benefit from surgery. Optimal surgery should be performed, but pneumonectomy should be avoided whenever possible. Adjuvant treatment is suggested to provide an added survival advantage.