Effective diagnosis and surgical management of primary signet ring cell carcinoma of the eyelid.

Primary signet ring cell carcinoma of the eyelid is an aggressive and rare neoplasm. It generally develops from the eccrine sweat gland and diffuses to the dermis and subcutis of the eyelid or axillae. The lesion usually presents as a progressive, diffuse and painless swelling of the eyelid with or without erythema and is frequently misdiagnosed as a chalazion or a chronic blepharoconjunctivitis leading to delayed management. The histology typically shows a signet ring cell or histiocytoid morphology. The authors present a case of a 76-year-old woman with a diagnosis of a primary signet ring cell carcinoma of the eyelid with atypical presentations. With this report, we seek to increase clinician awareness toward this tumor and to highlight the need for systematic recommendations in order to improve the management of these patients.

Flexor digitorum profundus tendon infiltration by post-traumatic hemangioma. A pediatric case.

Pediatric cases of post-traumatic hemangioma of the palm are rare. Clinical evaluation and imaging are sometimes ambiguous and surgical exploration may show more conclusive results. This case report discusses the diagnosis and treatment of a teenager with post-traumatic hemangioma infiltrating the flexor digitorum profundus tendon of the index finger. Pathologic examination revealed a lobular capillary hemangioma known as pyogenic granuloma.

Activity and safety of afatinib in a window preoperative EORTC study in patients with squamous cell carcinoma of the head and neck (SCCHN).

Background: To investigate the activity and safety of afatinib in the preoperative treatment of squamous cell carcinoma of the head and neck (SCCHN). Patients and methods: This study was an open-label, randomized, multicenter, phase II window of opportunity trial. Treatment-naïve SCCHN patients selected for primary curative surgery were randomized (5 : 1 ratio) to receive afatinib during 14 days (day -15 until day -1) before surgery (day 0) or no treatment. Tumor biopsies, 2-[fluorine-18]-fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET), and magnetic resonance imaging (MRI) were carried out at diagnosis and just before surgery. The primary end point was metabolic FDG-PET response (according to EORTC guidelines). Other end points included response assessment based on the Response Evaluation Criteria In Solid Tumors (RECIST) v1.1, dynamic contrast-enhanced (DCE)-MRI, diffusion weighted (DW)-MRI, safety, and translational research (TR). Results: Thirty patients were randomized: 25 to afatinib and 5 to control arm. Of the 23 eligible patients randomized to afatinib, 16 (70%; 95% CI: 47% to 87%) patients had a partial metabolic FDG-PET response (PMR). Five patients (22%; 95% CI: 8% to 44%) showed a partial response by RECISTv1.1. Responses assessed via DCE-MRI and DWI-MRI did not show a strong association with PMR or RECIST. One patient discontinued afatinib after 11 days for grade 3 diarrhea with subsequent renal failure and 24 days delay in surgery. No grade 4 toxicities or surgical comorbidities related to afatinib were reported. TR results indicated that PMR was more frequent in the tumors with high Cluster3-hypoxia score expression and with TP53 wild type. Conclusion: Afatinib given for 2 weeks to newly diagnosed SCCHN patients induces a high rate of FDG-PET partial metabolic response and partial response according to RECISTv1.1. Afatinib can be safely administered before surgery. Although exploratory, the hypoxic gene signature needs further investigations as a predictive biomarker of afatinib activity. Clinical trial registration: ClinicalTrials.gov: NCT01538381.

[A 38 years old female with inflammatory myofibroblastic tumor and lactic acidosis]. / Un cas de tumeur myofibroblastique inflammatoire maligne compliqué d'une acidose lactique chez une patiente de 38 ans.

Inflammatory myofibroblastic tumors (IMT) are rare tumors. They were originally described in the lung, but they have been now observed in many others locations, mainly abdominal and pelvic. These tumors are usually benign but their recurrent nature and the presence of an abnormality of chromosome band 2p23 in some of them, suggest that some lesions form a true tumor entity. Surgical excision as complete as possible is the gold standard treatment. We report the case of a 38 years old female, who presented a recurrent metastasizing inflammatory myofibroblastic tumor causing lactic acidosis and other biological abnormalities such as hypercalcemia, hypoalbuminemia, hypoglycemia, disseminated intravascular coagulation and inflammatory syndrome.

Gorham-Stout disease of the proximal fibula treated with radiotherapy and zoledronic acid.

Gorham-Stout disease is a rare disease characterized by anarchic lymphovascular proliferation causing resorption of bone sometimes leading to disastrous complications. Bone tissue is progressively replaced by angiomatic and lymphangiomatic tissue and finally by fibrous tissue. This disease is known to be ubiquitous and of complex etiology. We present a case of Gorham-Stout disease of the proximal fibula invading the proximal tibia and soft tissues of the popliteal space that was successfully treated with radiotherapy and zoledronic acid.

Intraperitoneal follicular dendritic cell sarcoma: role of chemotherapy and bone marrow allotransplantation in locally advanced disease?

We describe a case of a 44 year-old woman diagnosed with follicular dendritic cell sarcoma (FDCS). FDCS is a very rare disease affecting the dendritic antigen presenting cells and is often misdiagnosed. Surgery is considered the best treatment modality, followed by chemotherapy. In our case, surgical excision was not possible, therefore the patient received two lines of chemotherapy followed by bone marrow allotransplantation, then a third line of chemotherapy with a complete metabolic response seen on PET/computed tomography (CT) follow-up 29 months later. A review of the literature has been performed.

[Cardiac metastase of a soft tissue sarcoma: a case report and a review of the literature]. / Métastase cardiaque d'un sarcome des tissus mous: cas clinique et revue de la literature.

Soft tissue sarcomas represent rare tumors. They recur most often locally and in the lungs. We report the case of a 58-year woman who was treated for awith chemotherapy and then surgery. About a year after the end of the treatment an intracardiac mass was identified during a follow up chest CT-scan. The patient underwent a surgical resection of that mass found to bel myxoid chondrosarcoma metastasis. Only 2 cases of cardiac dissemination of extraskeletal myxoid chondrosarcoma have been described. The differential diagnosis of intracardiac masses is discussed. In some carefully selected cases atrisks of obstructive shock or embolization and unique metastatic location, cardiac surgery should be considered.

[Malignant solitary fibrous tumor of the abdominal wall in a man]. / Tumeur fibreuse solitaire maligne de la paroi abdominale chez un homme.

We report the first case of malignant solitary fibrous tumor of the abdominal wall in a man. Immunohistochemical staining for CD34 and bcl-2 were positive. Surprisingly, estrogen and progesterone receptors were focally positive. Expression of steroid hormone receptor in solitary fibrous tumor was rarely reported in the literature. In a few series, these receptors were identified as a risk factor of recurrences after surgical excision. Six months after complete surgical resection of the mass, our patient has been quite well without any evidence of recurrence.

Myxofibrosarcoma involving the external saphenous nerve: a case report.

Myxofibrosarcoma, also known as "myxoid malignant fibrous histiocytoma" is one of the most common soft tissue sarcomas in the extremities of adult patients. Most cases are characterized by a multinodular and/or infiltrative pattern with a tendency to infiltrate along fascial planes. We report a case of myxofibrosarcoma with an unusual presentation. Despite the clinical impression of a complete surgical resection, microscopic sarcomatous foci extended along the perineurium of the external saphenous nerve proximally up to 20 cm away from the main tumour. This unique case represents an extreme example of the locally agressive behaviour of soft tissue sarcomas and illustrates the microscopic tumour invasion along major nerves.

[Graft-versus-host disease: Review of 22 cases]. / Maladie du greffon contre l'hôte: revue de 22 cas.

PURPOSE: Graft-versus-host disease (GVHD) is one of the major complications of allogeneic bone marrow transplantation. Twenty-two patients, who had an allogeneic bone marrow transplantion at the Institute J. Bordet, developed a GVHD proven by a biopsy. RESULTS: Twenty-two cases of GVHD were registered; 17 of these patients suffered from the acute form of the disease. All the patients presented the characteristic skin lesions, usually associated with other organ involvement. Most of the cases suffered from relapse after a time-limited response or from resistance to therapy, despite an effective treatment. Uncontrolled GVHD led to the death of two patients. CONCLUSION: GVHD is a frequent pathology that significantly contributes to the morbidity and mortality associated with bone marrow transplantation, despite appropriate management. Recognition of clinical and histopathological features of GVHD is important for dermatologists involved in the care of bone marrow transplant patients. Actually, bone marrow transplantation is now easily and more frequently performed than in the past.

Trisomy 6 in Merkel cell carcinoma: a recurrent chromosomal aberration.

UNLABELLED: We retrospectively investigated 17 cases of primary and metastasizing Merkel cell carcinomas (MCC) from 14 patients using chromosomal in-situ hybridization (CISH) to study the occurrence of trisomy 6 in these lesions. METHODS AND RESULTS: Histological diagnosis on all tumour samples was obtained on haematoxylin and eosin stained sections. Immunohistochemistry was performed with antibodies against pancytokeratin (CAM 5.2), cytokeratin 20 (CK20), MIC2 antigen (CD99), neuron-specific enolase (NSE), and chromogranin A (chrA). Sections (4 microm) of the paraffin-embedded tumours were analysed with alpha-satellite centromeric probes for chromosome 6 or 17 using CISH. The signal was amplified by the Tyramide Signal Amplification (TSA) assay. Immunohistochemically, the tumours showed the same general epithelial neuro-endocrine pattern: 11/13 expressed cytokeratin 20, and 47% exhibited trisomy 6, with no significant difference between primary and metastatic lesions. Incomplete follow-up data did not allow us to establish a prognostic value of trisomy 6, however, this aberration might be an additional diagnostic tool in distinguishing MCC from other small round blue cell tumours. CONCLUSIONS: CISH seems to be a promising adjunctive method to diagnose Merkel cell carcinoma. Trisomy 6 should be investigated more closely in these cases, as has been done for chromosomes 1 and 11. Of particular interest would be identification of modifications in proto-oncogene(s) located on chromosome 6.

Development of a desmoid tumor at the site of a total hip replacement.

Extra-abdominal desmoid tumors are locally aggressive benign soft tissue tumors arising from the connective tissue of muscle and overlying fascia or aponeuroses. Location around the hip occurs in about 10% of all desmoid tumors. The authors report the development of a desmoid tumor around the hip 45 months after implantation of a total hip prosthesis. Endocrine and physical factors seem to play an important role in the development of the disease. Moreover, an association has been reported between trauma and desmoid tumor. Desmoid tumors developing around silicon implants have also been described. However, no association between hip prostheses and desmoid tumors has been published in the world literature. Although soft tissue tumors induced by metallic implants have been observed clinically, we still seek a coherent explanation for the exact pathogenesis.

Intramuscular myxoma.

Intramuscular myxoma is a rare, benign, mesenchymal tumour. It contains fibroblast-like, histiocyt-like and myofibroblast-like cells in a myxoid material. These tumours occur in adults between 40 and 60 years with a predilection for women (70%). The incidence varies between 0.10 and 0.13/100.000. They may occur as an independent lesion or in conjunction with fibrous dysplasia or Albright syndrome. Although only circumstantial features have linked fibrous dysplasia and myxoma, the concept of a basic metabolic error of both tissues during initial growth period has been proposed. There are no specific clinical manifestations of this tumour. Although MRI offers the best imaging approach in terms of determination of the local extension of the tumour and its fatty texture, the differential diagnosis between intramuscular myxoma and myxoid degenerescence of a liposarcoma must be considered. The diagnosis is always made after microscopic examination. Local excision with histological margins free of tumour provides excellent local control without recurrence. The case of a patient presenting this rare tumour is presented. A thorough survey of the literature was done.

[Anatomo-clinical conference: pulmonary cancer and cerebral vascular accident]. / Séance anatomo-clinique: cancer pulmonaire et accident vasculaire cérébral.

A patient with a lung adenocarcinoma died shortly after a first chemotherapy course from multifocal neurological lesions and severe cardiopathy. Autopsy has revealed the cause of these complications.

[Malignant hemangiopericytoma of the pelvis: treatment using internal hemipelvectomy]. / L'hémangiopéricytome malin du bassin: traitement par hémipelvectomie interne.

Hemangiopericytoma is a rare vascular tumor originating from pericytes. The main manifestation is a growing mass. Although computed tomodensitometry and MRI are the best diagnostic procedures, the diagnosis is made only after microscopic examination. The malignancy of this tumor is not well established. In fact, there is a continuum between benign and malignant hemangiopericytoma. The malignancy is estimated by the number of mitoses and the cellularity. Recurrences occur in 60% of cases. The best treatment is surgical excision. The role of radiation therapy and chemotherapy is not well established. The history of a 51-year-old woman treated by internal hemipelvectomy is presented here.