Breast implant-associated anaplastic large cell lymphoma: two distinct clinicopathological variants with different outcomes.

BACKGROUND: ALK-negative anaplastic large cell lymphoma associated with breast implant (i-ALCL) has been recently recognized as a distinct entity. Among 43 830 lymphomas registered in the French Lymphopath network since 2010, 300 breast lymphomas comprising 25 peripheral T-cell lymphomas (PTCL) were reviewed. Among PTCL, ALK-negative ALCL was the most frequent and all of them were associated with breast implants. PATIENTS AND METHODS: Since 2010, all i-ALCL cases were collected from different institutions through Lymphopath. Immuno-morphologic features, molecular data and clinical outcome of 19 i-ALCLs have been retrospectively analyzed. RESULTS: The median age of the patients was 61 years and the median length between breast implant and i-ALCL was 9 years. Most implants were silicone-filled and textured. Implant removal was performed in 17 out of 19 patients with additional treatment based on mostly CHOP or CHOP-like chemotherapy regimens (n = 10/19) or irradiation (n = 1/19). CHOP alone or ABVD following radiation without implant removal have been given in two patients. The two clinical presentations, i.e. effusion and less frequently tumor mass correlated with distinct histopathologic features: in situ i-ALCL (anaplastic cell proliferation confined to the fibrous capsule) and infiltrative i-ALCL (pleomorphic cells massively infiltrating adjacent tissue with eosinophils and sometimes Reed-Sternberg-like cells mimicking Hodgkin lymphoma). Malignant cells were CD30-positive, showed a variable staining for EMA and were ALK negative. Most cases had a cytotoxic T-cell immunophenotype with variable T-cell antigen loss and pSTAT3 nuclear expression. T-cell receptor genes were clonally rearranged in 13 out of 13 tested cases. After 18 months of median follow-up, the 2-year overall survival for in situ and infiltrative i-ALCL was 100% and 52.5%, respectively. CONCLUSIONS: In situ i-ALCLs have an indolent clinical course and generally remain free of disease after implant removal. However, infiltrative i-ALCLs could have a more aggressive clinical course that might require additional therapy to implant removal.

[Pyoderma gangrenosum revealing colonic diverticulitis: Two cases]. / Pyoderma gangrenosum révélant une diverticulite colique: deux cas.

BACKGROUND: The association of pyoderma gangrenosum (PG) with colonic diverticulitis infection (DI) is relatively unknown. Herein, we describe two cases of PG with full recovery after colonic surgery. PATIENTS AND METHODS: Case 1: an 83-year-old man presented with lesions on his legs that had been present for several weeks, and a diagnosis of PG was confirmed histologically. Abdominopelvic computed tomography (CT) performed on account of biological inflammatory syndrome revealed DI complicated by abscesses. Following the failure of two different antibiotic regimens, sigmoidectomy was performed. Postoperatively, the skin lesions healed without local or systemic corticosteroids. Case 2: a 63-year-old woman presented PG resistant to local and systemic corticosteroids and dapsone for several months. A particularly severe flare was accompanied by abdominal pain and inflammatory syndrome. CT revealed perforated sigmoid DI. Sigmoidectomy was performed after failure of drug therapy. The patient's PG subsequently improved and had disappeared without recurrence at 24months. DISCUSSION: Both of these cases of PG revealed DI. The hypothesis is that DI constituted a source of colonic inflammation, sending out bacterial antigenic stimuli that resulted in PG through deposition of circulating immune complexes. Removal of this inflammatory source appears to have enabled healing of PG. CONCLUSION: DI must be added to the list of systemic diseases associated with PG. In the case of isolated PG, CT may be used to detect asymptomatic DI. Early diagnosis could prevent serious gastrointestinal complications.

[Differential diagnosis between keloid and hypertrophic scars: a new approach by full-field optical coherence tomography]. / Diagnostic différentiel entre cicatrices chéloïdes et hypertrophiques : une nouvelle approche en tomographie par cohérence optique plein-champ.

OBJECTIVE: To evaluate the efficacy of full-field optical coherence tomography to differentiate hypertrophic and keloid scars on ex-vivo tissues. PATIENTS AND METHODS: All patients who underwent resection of pathological scar from November 2012 to September 2013 were analyzed. The scars were fixed in formalin and analyzed by conventional histology and full-field optical coherence tomography. The criteria for evaluation were: presence of dermal nodules, presence of cells and hyalinization of collagen. RESULTS: Nineteen pathological scars were analyzed. Histology found 7 keloid scars, 7 mixed and 3 hypertrophic scars. The sensitivity of optical coherence tomography for the detection of dermal nodules was 100%. This technology was not helpful for detection of cells and hyalinized collagen. CONCLUSION: In the present state of technology, optical coherence tomography did not identify the presence of cells, which makes the differential diagnosis difficult in the case of hypertrophic and keloid scars.

[Establishment of a keloid model by transplanting human keloid onto the backs of nude mice]. / Mise au point d'un modèle animal original de cicatrice chéloïde.

Keloid scar is a proliferative healing dysfunction formed by an excessive build-up of collagen fibers on the dermis. It is responsible of aesthetic and functional disabilities. There is no ideal treatment and recurrence occurs very often. Keloid scars occur only to human, that's why animal model needs to be made to study this pathology and new treatments. Few models have been described using human keloid scars implanted into subcutaneous tissue of nude mice or rat. To allow study of topical and laser treatment we have developed a new animal model using human keloid scar fragment with epidermal and dermal tissue implanted into back of nude mice like a full thickness skin graft. Keloid fragments from five donors have been grafted onto 40 nudes mice. Macroscopic and microscopic studies have been made at day 28, 56, 84 and 112. We observed integration of the fragments in all cases. Hyalinized collagen bundles were observed in all implant biopsies confirming the stability of the keloid architecture within 112 days. This model is easily reproducible and allows the study of topical treatment and laser due to the accessibility of the keloid.

[Abdominal cystic tumor revealing lymphangioleiomyomatosis]. / Tumeurs kystiques rétropéritonéales compliquées révélatrices d'une lymphangioléiomyomatose.

We report the case of a 39 year-old woman with many years of intermittent abdominal pain who was found to have cystic masses evocative of cystic lymphangioma involving the posterior mediastinal and retroperitoneum. Worsening abdominal pain led to a recommendation for laparoscopic unroofing and decompression of the cysts. During the postoperative period, hemorrhagic shock required reintervention with excision of the tumoral mass. Pathologic examination revealed lymphangioleiomyomatosis (LAM). On the 15th postoperative day, the patient developed a chylopneumothorax which required prolonged chest tube drainage. The presence of multiple polycystic lesions in the pulmonary parenchyma supported the diagnosis of diffuse LAM with primary extrapulmonary presentation. This diagnosis should be considered preoperatively since it modifies the treatment: a complete excision of the cystic lesions seems to be necessary in order to prevent bleeding and lymphatic extravasation.

c-Met overexpression in inflammatory breast carcinomas: automated quantification on tissue microarrays.

Inflammatory breast carcinoma (IBC) is a rare but aggressive tumour associated with poor outcome owing to early metastases. Increased expression of c-Met protein correlates with reduced survival and high metastatic risk in human cancers including breast carcinomas and is targetable by specific drugs, that could potentially improve the prognosis. In the present study, we compared c-Met expression in IBC (n=41) and non-IBC (n=480) immunohistochemically (Ventana Benchmark autostainer) in two tissue microarrays (TMA) along with PI3K and E-cadherin. The results were quantified through an automated image analysis device (SAMBA Technologies). We observed that (i) c-Met was significantly overexpressed in IBC as compared with non-IBC (P<0.001), (ii) PI3K was overexpressed (P<0.001) in IBC, suggesting that the overexpressed c-Met is functionally active at least through the PI3K signal transduction pathway; and (iii) E-cadherin was paradoxically also overexpressed in IBC. We concluded that overexpressed c-Met in IBC constitutes a potential target for specific therapy for the management of patients with poor-outcome tumours such as IBC. Automated image analysis of TMA proved to be a valuable tool for high-throughput immunohistochemical quantification of the expression of intratumorous protein markers.

[Difficult diagnosis of fever of unknown origin related to an infraclinic liposarcoma: "small tumor and fever of unknown origin"]. / Diagnostic difficile d'un liposarcome infraclinique responsable d'une fièvre au long cours : << petite tumeur, grande inflammation >>

INTRODUCTION: Palpation of soft tissues constitutes part of the physical examination when faced with a patient with fever of unknown origin. Our case highlights the difficulty of diagnosis when the tumor still remains impalpable ten months after onset of fever and severe biological inflammatory process. EXEGESIS: A 49-year-old woman was admitted for fever of unknown origin associated with important biological inflammatory process. In view of her past medical history of breast carcinoma, a search for a relapse was performed and remained negative. Despite multiple investigations and repeated physical examinations, no diagnosis was obtained. Finally, [18F] fluorodoexyglucose positron emission tomography led to a diagnosis showing a high fixation located in the right thigh, related to a myxoid liposarcoma after surgical resection. CONCLUSION: Dramatic regression of fever and biological inflammatory process after surgical treatment illustrates the concept of "small tumor and fever of unknown origin", an exceptional entity but which physicians should be aware of. The role of new technical imaging with [18F] fluorodoexyglucose positron emission tomography has however to be clarified in the evaluation of fever of unknown origin.

Sarcoidosis preceding chronic myelomonocytic leukemia. Report of two cases.

We report 2 patients who had sarcoidosis with skin involvement associated with bilateral anterior uveitis. During the follow-up, they developed chronic myelomonocytic leukemia. This association is of particular interest as sarcoidosis and chronic myelomonocytic leukemia may be considered as disorders of a common monocyte-macrophage cell lineage.

[A classical form of actinomycoses involving the mouth, face bones, orbit and base of the cranium in an African patient]. / Une forme historique d'actinomycose impliquant la cavité buccale, les os de la face, l'orbite et la base du crâne chez un patient africain.

Osteitis due to actinomycosis is now an uncommon clinical entity in industrialized countries. This report describes a classic case of imported actinomycosis involving the buccal cavity, facial bones, orbit and skull base. For two years after the onset, various diagnoses followed by different medical as well as surgical treatments were unsuccessfully attempted first in Senegal then in Italy. Proper diagnosis was finally established in Marseille, France, on the basis of histological findings after extensive surgical biopsy. The patient was treated with high-dose intravenous penicillin G for four weeks followed by daily oral administration of amoxicillin for one year. Surgical reconstruction could not be carried out before the patient's return to Senegal. The pitfalls of diagnosis and treatment of actinomycosis are discussed.

[Arguments in favor of adipocyte grafts with the S.R. Coleman technique]. / Plaidoyer en faveur de la greffe adipocytaire dans la technique de S.R. Coleman.

The main criticism against fat reinjection procedures is related to whether it is possible to graft adipocytes or not. The ideal solution would be to perform biopsies a few months after the operation to check the evolution of the grafted tissue, but such an approach would be difficult to accept for our patients. To overcome this difficulty the authors have compiled all the arguments that plead in favor of adipocyte grafts when Coleman's technique is used. Peer demonstrated in animal studies that it is possible to graft fatty tissue and that more resorption occurs when the fragments are large in size. The histologic studies have shown that the different stages of Coleman's technique do not alter the viability of the adipocytes. A disorganization of the architecture of the lobules is observed, but this does not compromise the theory of adipocyte grafting. An MRI study, performed on ten patients, demonstrated that the reinjected fat cells persist several months after the procedure. These fat cells presented the same characteristics as the patient's native fat in the surrounding area. This impression was confirmed by histological analysis of the reinjected fatty tissue 18 months after the graft. Finally, the clinical results obtained on over 200 patients treated for various indications in plastic and cosmetic surgery lead us to believe that it is possible to perform grafts of fatty tissue.

[Infiltrating syringoadenoma of the nipple: a new case]. / Syringoadénome infiltrant du mamelon: un nouveau cas.

Infiltrating syringomatous adenoma of the nipple is composed of small sweat ducts and solid strands, surrounded by desmoplastic stroma and preferentially develops in the superficial breast tissue and specially in the nipple. This particular and exceptional lesion deserves to be acknowlegded by pathologists concerned with breast pathology since this tumor mimics grade 1 invasive breast ductal carcinoma.

Clinicopathologic growth factors in vestibular schwannomas: a morphological and immunohistochemical study of 69 tumours.

Tumour growth of vestibular schwannomas is still difficult to predict. The aim of our study was to determine whether any defined histopathological feature was correlated with the clinical course. We did a retrospective study with 69 paraffin-embedded tumours to establish whether the number of vessels, blood cells extravasation or degree of inflammation, all semi-quantitatively assessed, could be indicative of potential of growth. An immunohistochemical study was also performed with an endothelial marker CD34, the leukocyte common antigen CD45 and the estrogen and progesterone hormone receptors. All these parameters were correlated with patient's age, duration of symptoms (d), with a clinical growth index (CLI = tumour size/d). No clinical parameters proved to be predictive of tumour growth. Tumour size was significantly (p = 0.01) related to the number of vessels and we found a significant relationship between the clinical growth index (CLI) and total number of vessels, especially when duration of symptoms lasted less than 1 year (p < 0.001). However, we found no relationship between duration of symptoms or CLI and CD34 index. The degree of inflammation was significantly correlated (p = 0.007) with duration of symptoms when it lasted more than 1 year. The CD45 index and the semi-quantitative evaluation of the inflammation were well correlated (p = 0.001). No estrogen receptors antigenic site was detected and only seven tumours expressed progesterone receptor in a few cells without any significant clinical value. These results suggest that vessel density is determinant for sporadic acoustic neuroma growth especially for a short clinical course.

[Retroperitoneal ectopic pregnancy: report of a case]. / Grossesse ectopique rétropéritonéale: à propos d'un cas.

Retroperitoneal space is an exceptional location of extrauterine pregnancy (four cases have been previously reported). The authors report a case of retroperitoneal ectopic pregnancy. Pathogenesis is discussed.

Early curettage of giant congenital naevi in children.

Early curettage of giant congenital naevi in new-born infants, if performed during the first few weeks after birth, can have a substantial effect on the pigmentation of the lesions, reducing the number of pigmented cells and leaving only moderate scars. We describe our experience in a series of nine congenital naevi treated by curettage between the first and seventh week after birth. Histological changes that occur in the naevus during the first few days after birth mean that early treatment is needed to obtain a cosmetic improvement. Curettage as an early treatment for giant congenital naevi in new-born infants provides cosmetic improvement and might decrease the risk of malignancy; however, the lesions are not cured. Naevus cells persist in the deep dermal layers and may come to the surface, such that close monitoring is needed long after curettage. The cosmetic improvement is likely to benefit the child's psychological development.

Desmoid tumour of the breast.

We describe the clinical and pathological features of a case of desmoid tumour of the breast. The lesion was approximately 3.0 cm in greatest dimension. Histologically, it had infiltrating borders and extended to the pectoral muscle. The differential diagnosis is discussed.

[Lymphocytic mastitis]. / Les mastites lymphocytaires.

Lymphocytic mastitis is a non infectious inflammatory disease of the breast with lobulocentric lymphocytic infiltrate of variable intensity, collagenous fibrosis and progressive lobular atrophy. The pathogenesis of lymphocytic mastitis is still unknown but some recent reports have suggested an autoimmune origin. We investigated a series of 10 cases by immunohistochemistry and we collected patients' biologic data. The most striking histologic feature was a prominent lobulocentric stromal or intraepithelial lymphocytic infiltrate. Occasionally, the infiltrate was perivascular and nodular along the lobule border. B and T lymphocytes, both demonstrated by immunophenotypic analysis, were shown with a particular pattern of distribution. Pathologists must be aware of this disease in order to recommend immunological investigation.

[Anetoderma during antiphospholipid syndrome. 3 cases]. / L'anétodermie au cours du syndrome des antiphospholipides. Trois cas.

INTRODUCTION: Anetoderma is a skin disease of unknown etiology sometimes encountered in patients with a lupus syndrome. We report a clinical and pathology analysis of 3 selected cases of anetoderma associated with systemic lupus erythematosus and/or antiphospholipid syndrome. CASE REPORTS: Three patients had skin lesions typical of anetoderma. Antiphospholipid antibodies were present in all three (anticardiolipin in 3, circulating lupus anticoagulants in 3, anti-beta 2GP1 in 2). One patient with systemic lupus erythematosus had signs of antiphospholipid syndrome, the two others had primary antiphospholipid syndrome. DISCUSSION: The analysis of our cases and a review of the literature suggest that anetoderma is a disease which develops in the immunological environment of lupus, and is apparently more related to the presence of antiphospholipids than lupus itself. Thus anetoderma could be another cutaneous sign of the antiphospholipid syndrome.