[Effect of surgery of pulmonary cysts related to immunoglobulin light chain deposits]. / Intérêt de la chirurgie de kystes pulmonaires liés à des dépôts de chaînes légères.

INTRODUCTION: Light chain deposition disease is a rare anatomo-clinical disorder, which rarely leads to cystic lung destruction. CASE REPORT: We report the case of a 62years old female patient with a history of a monoclonal gammopathy of unknown significance who developed progressive dyspnea. Thoracic CT-scan demonstrated a diffuse pulmonary cystic disorder with predominance in the right lower lobe. Thoracoscopic surgical resection of that lobe led to a diagnosis of non-amyloid kappa light chain deposits. Surgery also resulted in a lung volume reduction effect with clinical and functional benefits related to improved ventilation of adjacent segments. CONCLUSION: This report of pulmonary cystic disorder related to a light chain deposition disease highlights the potential clinical and functional benefits observed after lung volume reduction surgery.

[Anatomoclinical and immunohistochemical study of hidradenoma papilliferum, a tumor deriving from anogenital mammary-like glands]. / Étude anatomoclinique et immunohistochimique d'une tumeur dérivant des glandes ano-génitales de type mammaire : l'hidradénome papillifère.

AIM: To specify the clinical, histopathological and immunochemical features in a large series of hidradenoma papilliferum patients and to point out similarities with breast disease. PATIENTS AND METHODS: We performed a monocentric retrospective histopathological study. Cases of hidradenoma papilliferum seen at the dermatopathology laboratory of Strasbourg (1977-2017) were included and analyzed with hematoxylin-eosin staining and immunostaining. Diagnosis was confirmed where tubular formations and papillary proliferations were noted and were lined by a luminal layer of columnar cells surrounded by a basal layer of myoepithelial cells. RESULTS: We included 32 cases solely involving women of a mean age of 49 years. Most cases involved the vulva (67 %) or the perianal region (25 %). The lesions ranged in size from 3 to 25mm. They occurred as solitary, non-ulcerated, skin-colored and cyst-like lesions. They were mostly localized in the mid-dermis. We identified several peculiar morphological variants. Three HP had a structure resembling tubular apocrine papillary adenoma, 3 were similar to apocrine cystadenoma, while 9 HP, of which 3 involved epidermal hyperplasia, were connected with the overlying epidermis and had dense stromal infiltrate in plasma cells, closely resembling syringocystadenoma papilliferum. Sixteen tumors had no features of note. Similarities with breast tumors were revealed through columnar cell hyperplasia (6 cases), high mitotic rates (4 cases), and atypical nuclei (3 cases). Anogenital mammary-like glands were found in the immediate vicinity of 10 HP. Myoepithelial cells were immunoreactive for smooth muscle actin and p63, while luminal cells were immunoreactive for CK7, CK8, EMA, and, in a more focused and less consistent manner, for Ber-EP4, CD117, PHLDA1 and androgen receptors. CONCLUSION: Our study highlighted a variety of patterns and cell morphologies, on occasion within a single tumor, identical to breast disease. Histological and immunochemical examination revealed that anogenital mammary-like glands and HP share common features, which militates in favor of a common histogenesis.

[Mesenteric lymph node cavitation in celiac disease: Report of four cases and literature review]. / Le syndrome de cavitation ganglionnaire mésentérique, complication rare de la maladie cœliaque de l'adulte : à propos de quatre cas et revue de la littérature.

INTRODUCTION: Mesenteric lymph node cavitation is an exceptional complication of celiac disease. We report four original observations of this syndrome, completed by a literature review. DISCUSSION: The analysis of 38 cases showed that this complication occurred exclusively in adults, with a mean age at diagnosis of 54 years. It revealed the celiac disease in the majority of cases. Hyposplenism was almost systematically associated. The risk of lymphoma appeared higher, especially enteropathy-associated T-cell lymphoma. The prognosis was poor with nearly 50% mortality and seemed related to the clinical response to the gluten-free diet. CONCLUSION: The severity of this complication deserves to be known and should lead to its research in celiac patients, especially in cases diagnosed in adulthood or in case of refractory disease.

Consistency in recognizing microinvasion in breast carcinomas is improved by immunohistochemistry for myoepithelial markers.

Microinvasion is the smallest morphologically identifiable stage of invasion. Its presence and distinction from in situ carcinoma may have therapeutic implications, and clinical staging also requires the recognition of this phenomenon. Microinvasion is established on the basis of several morphological criteria, which may be difficult and not perfectly reproducible among pathologists. The aim of this study was to assess the consistency of diagnosing microinvasion in the breast on traditional haematoxylin and eosin (HE) stained slides and to evaluate whether immunohistochemistry (IHC) for myoepithelial markers could improve this. Digital images were generated from representative areas of 50 cases stained with HE and IHC for myoepithelial markers. Cases were specifically selected from the spectrum of in situ to microinvasive cancers. Twenty-eight dedicated breast pathologists assessed these cases at different magnifications through a web-based platform in two rounds: first HE only and after a washout period by both HE and IHC. Consistency in the recognition of microinvasion significantly improved with the use of IHC. Concordance rates increased from 0.85 to 0.96, kappa from 0.5 to 0.85, the number of cases with 100% agreement rose from 9/50 to 25/50 with IHC and the certainty of diagnosis also increased. The use of IHC markedly improves the consistency of identifying microinvasion. This corroborates previous recommendations to use IHC for myoepithelial markers to clarify cases where uncertainty exists about the presence of microinvasion. Microinvasive carcinoma is a rare entity, and seeking a second opinion may avoid overdiagnosis.

Successful lung retransplantation in a patient with acute fibrinous and organizing pneumonia: a case report.

Acute fibrinous and organizing pneumonia (AFOP) is an unusual histopathologic pattern characterized by the formation of intra-alveolar plugs of fibrin deposition and associated organizing pneumonia. AFOP is considered to be a form of rejection and portends a dismal prognosis. Here, we present the case of a young male patient who initially underwent a double lung transplantation for cystic fibrosis. After 42 months of regular follow-up, he experienced rapidly progressive respiratory failure. Acute rejection and opportunistic lung infections were suspected. The clinical conditions rapidly deteriorated despite treatment with broad-spectrum antibiotics and high-dose steroids. Therefore, AFOP was suspected owing to: 1) acute clinical presentation; 2) pulmonary computerized tomographic data; 3) typical histopathologic findings on transbronchial biopsieseconds, and 4) lack of response to different treatments. The patient required an emergency bilateral lung retransplantation 44 months after the initial transplantation. The histopathologic analysis of the explanted lungs confirmed the diagnosis of AFOP. Two years after the 2nd transplant, the patient is alive and well. To the best of our knowledge, this is the 1st case of a patient experiencing AFOP following lung transplantation who was successfully rescued by a 2nd bilateral lung retransplantation.