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Alveolar soft part sarcoma (ASPS) is a rare mesenchymal soft-tissue tumour. It commonly arises in the lower extremities in adults and the head and neck in children. Primary pulmonary involvement, without evidence of soft tissue tumour elsewhere, is very rare. We present the seventh case ever reported in the literature of primary ASPS of the lung, which was revealed by brain metastases in a 28-year-old male patient. The parietal brain tumour was resected. The radiological and histological study led to the diagnosis: metastasis of an alveolar sarcoma. The patient then had brain radiotherapy and chemotherapy. Using this case as a starting point, we reviewed the characteristics and the main therapeutic options in these uncommon neoplasms.

Lumbar pigmented villonodular synovitis is rarely localized on the lumbar spine. Awareness of pigmented villonodular synovitis is important despite its rarity because it may mimic several other lesions of this location.

Tuberculosis (TB) is one of the most important infectious diseases, particularly in the world. Amongst the genitourinary organs, prostatic TB is less common. We report an 68 year old patient, immunocompetent who presented obstructive and irritative symptoms of the lower urinary tract. A history of pulmonary tuberculosis was not present. The digital rectal examination was suspicious and PSA was a normal. The biopsy results did not reveal any malignant lesions but the transurethral resection of the prostate performed for voiding purposes showed prostatic tuberculosis. A very good clinical and biological improvement was observed after chemotherapy anti-tuberculosis.

Background:Bullous pemphigoid (BP) is the most common autoimmune bullous disease, which is usually seen in older ages. Rarely, radiotherapy (RT) has been reported as a localised trigger factor for BP.Case:We present a case of a 64-year-old woman who underwent radical mastectomy and radiation therapy for breast cancer. Two months after the completion of RT with adjuvant Trastuzumab, a BP eruption developed in the irradiated area, spreading thereafter to other skin areas. The patient was treated with oral corticosteroids and the lesions evolved favourably.Conclusion:This case demonstrates a relatively benign skin reaction that could develop during the course of cancer treatment, which required co-management by oncologists, pathologists and dermatologists.

Human hydatid disease is still endemic in pastoral and rangeland areas, with temperate climate, mainly in the southern shore of the Mediterranean, particularly in the Maghreb countries. Renal localization is rare although it is the most frequent site of the urinary tract. Its clinical evolution remains silent for long time, and the diagnosis is often elusive for years. Renal hydatid cysts may pose a problem of differential diagnosis. Our case concerns a renal hydatidosis misdiagnosed as a xanthogranulomatous pyelonephritis, treated by total nephrectomy.

Introduction and importance Hidradenitis suppurativa (HS) is a chronic inflammatory and suppurative disease of the apocrine sweat glands. Its transformation into squamous cell carcinoma (SCC) is very rare. Case presentation We describe 3 cases of males aged 57, 58 and 55-years-old. The diagnoses were confirmed by pathology examination in all cases. Two of our patients underwent extended and complete surgery. The first two patients died during the year after the diagnosis was established, the third one is still alive with no recurrence after one year of surgery. Clinical discussion Hidradenitis suppurativa mostly concerns perianal location and it targets mainly male patients with a multifactorial development. Surgical treatment consists of large excision. The SCC is associated with a high mortality rate. Conclusion Hidradenitis suppurativa needs early diagnosis and effective surgery. The transformation into squamous cell carcinoma is rare and and its management is challenging. Extended and complete excision is required with rigorous postoperative follow-up., Highlights • The transformation of Hidradenitis suppurativa into squamous cell carcinoma (SCC) is scarce. • The management of this uncommon disease is challenging and includes a combination of surgical and medical approaches. • Squamous cell carcinoma (SCC) is associated with a high mortality rate.

Atypical teratoid/rhabdoid tumor (AT/RT) represents a rare malignant embryonic tumor of infant and early childhood. Its prognosis remains dismal despite aggressive multimodal treatment. We report the case of a 24-year-old male who was diagnosed with left parietal AT/RT after total resection and who is still in good health and recurrence free 4 years after surgery and adjuvant chemotherapy and radiotherapy.

Bladder metastasis of cutaneous malignant melanoma is an extremely rare condition, with less than 10 cases reported in the last 30 years in the English literature. Bladder localization is most often asymptomatic, explaining the frequency of cases discovered during autopsy in multi-metastatic patients. We report a case of symptomatic malignant melanoma metastasis to the bladder in a 31-year-old patient.

Congenital gliosarcoma are a very rare entity and only 9 congenital gliosarcoma cases have been reported in the literature. It’s a variant of glioblastoma multiforme. Prognosis, however, sometimes is in contradiction with the pathological appearance and seems to be more closely related to the grade of surgical resection. This report documents the presentation of a 5-month-old boy with voluminous congenital gliosarcoma of the right frontal lobe. We describe clinical diagnosis, histological aspects, radiological features and current management of this very aggressive tumor. International consensus is yet to be envisaged for the management of this very aggressive tumor, with a special emphasis on antenatal detection of these cases.

Enormous bilateral renal angiomyolipoma (AML) are extremely rare, their spontaneous bleeding also called Wunderlich's syndrom (WS) is also an unusual situation.It is considered as a life threatening condition requiring fast and effective care.We present a case of hypovolemic shock due to spontaneous rupture of bilateral giant angiomyolipomas in a 35-year-old female patient with tuberous sclerosis complex (TSC).The hemodynamic instability of the patient leads to an immediate surgery and unilateral nephrectomy was done for the biggest angiomyolipomas. The review of the literature revealed only few cases of spontaneous rupture of renal angiomyolipomas of comparable size. Keywords: Wunderlich syndrom, Hematuria, Radical nephrectomy, Giant angiomyolipoma

Cauda equina paragangliomas are rare neuroendocrine benign and slow-growing tumors. Acute paraplegia, occurring because of sudden intratumoral hemorrhage, represents an extremely rare clinical picture of this disease. We report the case of a 64-year-old male presenting with a 5-day acute lower back pain, sciatica, and leg weakness. Spinal imaging showed an intradural mass of the cauda equina region and an emergent surgical treatment was achieved. The lesion was removed “en bloc,” and subarachnoid blood was noticed during surgery. The postoperative course was uneventful, with complete regression of pain and progressive motor recovery. The histological study revealed typical microscopic and immunohistochemical features of paragangliomas.

We present an illustrative case of pediatric intracranial anaplastic ganglioglioma and systematically reviewed the current reported data of anaplastic ganglioglioma in the pediatric population.A comprehensive literature search for our review was conducted using PubMed, Scopus, Web of Science, PsycINFO, Cochrane, and Embase databases. The search terms included "ganglioglioma," "anaplastic," "pediatrics," "children," and "intracranial." We used no limitations for date, type, or language. Reports of pediatric patients (age19 years) with intracranial anaplastic gangliogliomas were included. Baseline patient demographic characteristics, clinical presentations, imaging characteristics, management strategies, and outcomes data were extracted.We included 24 studies involving 34 patients in the quantitative synthesis. The mean patient age was 9.18 ± 5.46 years (range, 0.16-18). A male predominance was observed (approximate male/female ratio, 1.61:1). Increased intracranial pressure was the most common symptom (n = 19; 55%), followed by seizures (n = 11; 32%). These tumors were most often found in the supratentorial compartment (n = 26; 76%). All 34 patients had undergone surgical removal. The mean follow-up was 22.2 months (range, 2-72). The mean overall survival duration was 43 months (95% confidence interval, 32.45%-55.31%; 1- and 3-year overall survival, 76.6% and 45.5%, respectively). The median event-free survival was 34 months (95% confidence interval, 10.6%-57.3%; 1- and 3-year event-free survival rate, 76.6% and 42.4%, respectively).Our results contribute to our understanding of the characteristics of this rare malignant tumor and show that anaplastic ganglioglioma should be considered in the differential diagnosis of intracranial tumors in pediatric patients.

Background Pancreatic ductal adenocarcinoma (PDAC) has a devastatingly poor prognosis. Surgical resection is undertaken in only 20% of patients. Most of well-known prognostic factors reflect tumor stage more than its biology. So it is important to identify new biological indicators related to survival in order to develop new therapies. Objective To determine the relation between tumor budding and Epithelial Mesenchymal Transition (EMT) and to evaluate their impact on survival for patients after resection of PDAC. Methods We herein report a retrospective study of 50 patients with resected PDAC. Tumor budding, immunohistochemical expression of vimentin and other standard factors were correlated with survival using the Kaplan-Meier method and Cox multivariable survival analysis. For tumor budding assessment, an inter-observer variability study was performed using 100 images of tumor slides stained with Hematoxylin & Eosin and Pan-Cytokeratin. Results Tumor budding was present in all tumors. A substantial agreement between six pathologists was established in distinguishing high-grade from low-grade budding (κ = 0.6 and 0.73 for H&E and PCK images respectively). High-grade budding was identified in 56% of tumors (28/50). It was an adverse prognostic factor independent of tumor size, resection margins status, nodal status and vascular invasion (p = 0.008). Tumor budding was significantly associated with vimentin expression (p = 0.002). Conclusions The association of tumor budding with vimentin expression supported the idea that EMT is a key process in PDAC responsible for progression and drug resistance. Consequently, the elucidation of EMT molecular biology and development of new targeted therapy may improve disease outcome.

Highlights • Spinal epidural angiolipoma is a rare cause of progressive spinal cord compression. • Magnetic resonance imaging is necessary for diagnosis. • Total resection is the treatment of choice. • Necessary precautions before and during surgery have to be taken (embolisation, transfusion). • Prognosis after surgical management is excellent even at the stage of complete paraplegia., Introduction Angiolipomas are benign, slow growing lesions, almost always located subcutaneously in the trunc or limbs. They are composed of mature lipocytes admixed with abnormal blood vessels. Spinal epidural angiolipoma are rare accounting for approximately 0.14–1.2% of all spinal axis tumors and 2–3% of epidural spinal tumors. Presentation of case We report the case of a 65 years-old-woman, presenting with complete paraplegia installed since 7 months. Magnetic resonance imaging (MRI) showed an epidural dorsal fatty mass. The patient recovered immediately after surgery. The pathological examination concluded to an angiolipoma. Discussion Angiolipoma patients most commonly have long-lasting pain and then develop progressive neurological symptoms secondary to spinal cord compression. The mean duration of symptom progression at diagnosis is 1 year. MRI is the most reliable examination for the diagnosis of spinal angiolipoma. Total resection is the treatment of choice. No adjuvant treatment is indicated. Since SAL are very haemorrhagic lesions, preoperative embolization is recommended. Conclusion We think that spinal cord compression caused by angiolipoma have very good functional prognosis, even if tardily diagnosed

Objectif Etudier l’apport de l’examen extemporane dans la prise en charge des nodules thyroidiens et etablir une correlation avec les donnees preoperatoires et l’examen histologique definitif. Materiels et methodes Etude retrospective portant sur 130 patients ayant eu une thyroidectomie avec un examen extemporane puis un examen anatomopathologique definitif, colliges sur une periode de deux ans (janvier 2017–decembre 2018). Resultats L’âge moyen etait 48,7 ans avec un sex-ratio de 0,1. L’examen extemporane avait une valeur diagnostique statistiquement significative. Cette performance diagnostique etait satisfaisante en termes de specificite (100 %), sensibilite (93,3 %), valeur predictive positive (100 %) et valeur predictive negative (98,9 %). L’efficacite diagnostique etait 85,4 %. Les criteres epidemiologiques et cliniques avaient une sensibilite qui ne depasse pas 47,7 %, une specificite qui varie de 75,7 a 100 % et une efficacite diagnostique qui varie de 70,7 a 86,1 %. Les criteres echographiques avaient une sensibilite qui varie entre 14,2 et 61,9 %, une specificite entre 50,4 et 97,2 % et une efficacite diagnostique qui varie entre 44,6 et 83,6 %. La cytoponction avait une sensibilite de 80 %, une specificite de 90 % et une efficacite diagnostique de 51,8 %. Discussion En le comparant aux donnees preoperatoires cliniques, echographiques et cytologiques, l’examen extemporane dans la pathologie thyroidienne a prouve sa superiorite en termes de valeur diagnostique. Cependant, l’apport de cet examen reste limite par les diagnostics differes pour les microcarcinomes et les tumeurs d’architecture microvesiculaire.

Resume Objectifs Evaluer la valeur du coefficient apparent de diffusion (ADC) dans la prediction du grade et du sous-type des meningiomes ainsi que la differenciation entre meningiome de bas grade (grade I) et meningiome de haut grade (grades II et III). Patients et methodes Etude retrospective incluant 49 cas de meningiomes d’âge moyen de 50,7 ± 12,7 ans, repartis en grade I (n = 35), grade II (n = 11) et grade III (n = 3). Parmi les 35 meningiomes de grade I, 23 etaient meningotheliaux, 8 fibroblastiques, 3 transitionnels et 1angiomateux. Parmi les 11 meningiomes de grade II, 10 etaient atypiques et 1 a cellules claires. Parmi les 3 meningiomes de grade III, 2 etaient anaplasiques et 1 de type papillaire. Nous avons mesure la valeur la plus basse d’ADC au sein de la portion solide de la tumeur (ADCmin). Resultats Il existe une difference statistiquement significative entre les valeurs d’ADCmin mesurees dans les 3 groupes des meningiomes de grades I, II et III (p = 0,001) ainsi qu’entre les meningiomes de bas grade et de haut grade (p Conclusion L’ADC est un parametre utile, mais peu sensible, pour differencier les meningiomes de bas grade de ceux de haut grade. La baisse de l’ADC est en faveur d’un meningiome de haut grade.

Basal cell carcinoma (BCC) is the prototypical basaloid tumor of the skin. It may show various patterns simulating other cutaneous tumors due to its pleomorphism. It may have an unusal pattern of differentiation such as squamous, sebaceous, apocrine, eccrine, pilar, and endocrine differentiation. In order to establish the relative frequency of neuroendocrine differentiation in BCC, we performed a retrospective study of 33 consecutive BCCs using conventional immunohistochemistry with two neuroendocrine antibodies: Chromogranine A and synaptophysine. The age of the patients ranged from 17-83 years with mean of 65 years. The male to female ratio was 16:17. In immunohistochimestry, Chromogranine A was seen in 72.2% (24/33) while Synaptophysine was positive in 9.09% (3/33). Their expression was cytoplasmic and membranous and was seen in the periphery of these tumors in the overlying cells. Positive staining of chromogranine A was high (75-100% of tumors cells) in 9%, intermediate (25-75% of tumors cells) in 33% of cases and relatively low (

Primary retroperitoneal non Hodgkin lymphoma is extremely rare, its diagnosis is often difficult and it may requires a time consuming and a costly diagnostic workup. We report the case of a 46-year-old patient complaining of abdominal fullness and dorsal pain, who was diagnosed with an extra-nodal non-Hodgkin lymphoma presenting as a unique and large retroperitoneal mass. The suggested diagnosis was a malignant retroperitoneal tumor and the patient underwent an excision of the tumor throw a lombotomy followed by an R-CHOP chemotherapy regimen with good outcome. Keywords: Lymphoma, Large B-cell lymphoma, Diffuse lymphoma, Retroperitoneal extra-adrenal mass