[Recurrence of pseudomyxoma peritonei with liver and splenic metastases: about a case and literature review]. / Récidive d'un pseudomyxome péritonéal avec métastases hépatique et splénique: à propos d'un cas rare avec revue de littérature.

Pseudomyxoma peritonei (PMP) is a clinicopathologic syndrome characterized by mucinous ascites and pools of mucin resulting in neoplastic mucinous epithelium in the peritoneal cavity. PMP is uncommon and it is characterized by clinical and unusual pathologic manifestations posing diagnostic and therapeutic problems. Involvement of abdominal viscera and lymph node metastases are rare and sporadic cases have been reported in the literature. We here report the case of a 56-year old patient who had undergone two operations for PMP of appendicular origin presenting with progressive abdominal pain five years after his last treatment. Scanner objectified a recurrence of peritoneal pseudo-myxoma with liver and splenic intraparenchymatous lesions. Anatomopathological examination showed intrasplenic and hepatic recurrence of low-grade peritoneal pseudo-myxoma confirming the metastases.

[Uncommon mammary-type myofibroblastoma unlike any other: about a case]. / Un myofibroblastome de type mammaire pas comme les autres: à propos d'un cas.

Mammary-type myofibroblastoma (MTMF) is a rare benign mesenchymal tumor, initially detected in the breast. Its diagnosis is based on a spectrum of clinical, radiological and possibly histological parameters. We here report the rare case of a 50 year old male patient with mammary-type myofibroblastoma located in the axillary region. The peculiarity of our case lies in the presence of numerous mitoses and this was rarely described in the literature. The aim of our case study was to raise the problem of differential diagnosis of this rare tumor.

[Pneumoblastoma in adults: a new case report and literature review]. / Pneumoblastome de l'adulte: rapport d'un nouveau cas et revue de la litterature.

Pulmonary blastoma is a rare tumor which has been mainly reported in children. It accounts for 0.25-0.5% of all pulmonary tumors, with a very serious prognosis. Histologically, it is a tumor composed of two components: an epithelial component and a mesenchymal component. Clinically, it usually manifests as chest pain, cough, hemoptysis and dyspnea, but it is asymptomatic in approximately 40% of cases. We report the case of a 25 year old woman, with no previous medical history, who complained of dyspnoea, cough and left basithoracic pain. Radiological evaluation showed large basithoracic mass in the left lung. A biopsy was performed which only showed necrotic material. The surgical specimen was largely necrotic. The viable tissue was examined with the miroscope which showed biphasic pattern composed of malignant epithelial tissue associated with malignant mesenchymal tissue, typical of biphasic pneumoblastoma. The patient underwent chemotherapy and radiation therapy. Follow-up examination showed a recurrence, thus the patient underwent second line chemotherapy.

Synchronous adenomyomas of the ileum in an adult-an exceptional cause of intussusception.

In this article, we report a case of two synchronous ileal adenomyomas leading to intussusception. This rare occurrence has never been reported in the literature. Our case is noteworthy, because the lesion is rare and should be considered in the differential diagnosis of intussusception in adults.

Malignant renal epithelioid angiomyolipoma associated with abdominopelvic hydatid cysts: a case report.

INTRODUCTION: The World Health Organization defines epithelioid angiomyolipoma as a potentially malignant mesenchymal neoplasm characterized by proliferation of predominantly epithelioid cells and as closely related to the triphasic (classic) angiomyolipoma. It can be benign, potentially aggressive or malignant. The pathologist's role is crucial in making a positive diagnosis, providing appropriate patient management and assessing prognosis. In this report, we present a case of a patient with an epithelioid angiomyolipoma and hydatid cyst association. To the best of our knowledge, such an association has not been reported previously in the literature. CASE PRESENTATION: A 70-year-old Arabian woman presented to our hospital with a 6-month history of a right lumbago and weight loss. Computed tomography objectified a mid-right renal tumor, several locoregional lymph nodes and four abdominopelvic cystic formations. The patient underwent a right nephroureterectomy and removal of abdominal and pelvic masses. Histologically, the tumor corresponded to a proliferation of large eosinophil cells, polygonal or ovoid, with epithelial appearance, and associated with thickened, hyalinized vessel walls, fat cells and bundles of smooth muscle cells. Mitoses were estimated at 2 per 50 high-power fields. In immunohistochemical study, epithelioid tumor cells expressed S-100 protein and Melan-A. The diagnosis of malignant epithelioid angiomyolipoma was made. The wall of the abdominopelvic cysts was eosinophilic and lamellar, corresponding to the cuticular membrane of hydatid cysts. CONCLUSION: In our patient, careful histological examination and immunohistochemical study allowed us to make the correct diagnosis of angiomyolipoma in its malignant form. The association with hydatid cysts is what makes our case original.

Combined myoepithelial carcinoma and myoepithelioma in soft tissue: a case report and review of the literature.

INTRODUCTION: Soft tissue myoepithelial carcinoma and myoepithelioma are rare entities, part of myoepithelial tumors. They were incorporated into the World Health Organization classification of soft tissue tumors in 2002. Here we present an exceptional case of myoepithelial carcinoma and myoepithelioma association. To the best of our knowledge, such an association has never been reported in the literature. CASE PRESENTATION: We report a case of myoepithelial carcinoma combined with myoepithelioma occurring in the soft tissue of the right forearm of an 84-year-old Arabian man. We describe the clinical, radiological and pathological features dominated by histological polymorphism. We will also describe the proposed histological criteria of malignancy and the major role of immunohistochemistry in positive and differential diagnosis. We finally mention the therapeutic arsenal available. CONCLUSION: Through this work, we report that myoepithelioma of soft tissue can progress to malignant myoepithelioma.

Juxtaglomerular cell tumor: a distinct mesenchymal tumor of kidney.

Juxtaglomerular cell tumor (JGCT) is an unusual mesenchymal entity of the kidney. It is a benign renin-secreting tumor causing hypertension and hypokalemia due to secondary hyperaldosteronism. It is curable if it is discovered early and surgically removed, but may cause a fatal outcome usually due to complications of associated hypertension.

Non-traumatic myositis ossificans circumscripta: A diagnosis trap.

Myositis ossificans circumscripta (MOC) is a benign condition of non-neoplastic heterotopic bone formation in the muscle or soft tissue. Trauma plays a role in the development of MOC, thus, non-traumatic MOC is very rare. Although MOC may occur anywhere in the body, the lesions are localized predominantly in the high-risk sites of injury, such as the thigh, buttock, and elbow. MOC can easily be mistaken for osteomyelitis or a malignant tumor, specifically osteosarcoma or soft-tissue sarcoma. We report a rare case of non-traumatic myositis ossificans circumscripta of thigh which appear clinically and radiologically as a malignant neoplasm. Despite its rarity, MOC should be contemplated in the differential diagnosis of malignant tumors.

Renal myelolipoma: a rare extra-adrenal tumor in a rare site: a case report and review of the literature.

INTRODUCTION: Myelolipomas are uncommon, benign tumors composed of mature adipose tissue and hematopoietic elements. They mostly occur in the adrenal glands, but extra-adrenal myelolipomas have also been reported in other locations such as the presacral region, retroperitoneum, pelvis and mediastinum. Here, we present a case of an extra-adrenal myelolipoma in a rare site: the renal parenchyma. To the best of our knowledge, it is only the third case reported in this unusual location. CASE PRESENTATION: We report a case of primary myelolipoma occurring in the kidney of a 55-year-old Moroccan man. We describe the radiological and clinicopathologic features of this unusual tumor with a review of the literature, and we discuss differential diagnosis of retroperitoneal myelolipomas. CONCLUSION: This case is noteworthy because the tumor site was unusual. Although renal myelolipoma is rare, it should be considered in the differential diagnosis of lesions in this site.

A gallbladder tumor revealing metastatic clear cell renal carcinoma: report of case and review of literature.

Metastatic renal cell carcinoma in the gallbladder is extremely rare, with reported frequencies of less than 0.6% in large autopsy reviews. Only 40 cases were reported in the literature. We report a first case of gallbladder polypoid tumor revealing metastatic clear cell renal cell carcinoma, which demonstrates the importance of radiological tests, histology and immunohistochemistry when making a definitive diagnosis. These examinations also allow differentiating metastatic clear cell renal cell carcinoma from other polypoid lesions in the gallbladder with clear cell morphology. Cholecystectomy should be performed to obtain a definitive diagnosis and to improve survival in case of solitary metastatic renal cell carcinoma. VIRTUAL SLIDES: The virtual slides' for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/8956897238238989

Primary endobronchial leiomyosarcoma of the lung: clinical, gross and microscopic findings of two cases.

Primary leiomyosarcoma of the lung is an unusual malignant tumor. Among this entity, the endobronchial form is very rare and the preoperative diagnosis is extremely difficult. We present two different presentations and outcomes of primary endobronchial leiomyosarcoma of the lung. In both cases, the histological study and the immunohistochemical stain, of the surgical resection, provided the final diagnosis. Through those cases we present the diagnostic and therapeutic difficulties encountered.

Calcified multilocular thymic cyst associated with thymoma: a case report.

INTRODUCTION: There are few case reports of thymoma with a thymic cyst. Such an association renders it difficult for any pathologist to differentiate from other neoplasms, such as a cystic thymoma. CASE PRESENTATION: A 50-year-old Berber woman from Morocco was admitted with a chronic cough of more than 10 years duration. Her medical history and physical examination were normal. Anterior chest radiography demonstrated a calcified opacity in her right anterior mediastinum. A chest-computed tomogram revealed a round cystic tumor, with significant calcification in her right anterior mediastinum. A surgical exploration was performed. The tumor seemed to be a well-encapsulated and totally calcified lesion, arising from the right lobe of her thymus. It was removed by partial resection of her thymus. Through histology, the calcified tumor exhibited some areas of multilocular fibrous-wall cysts. These cysts were partially lined by small cuboidal cells with severe chronic inflammation and an AB thymoma that arose from the wall of the cyst. CONCLUSION: Greater attention should be given to multilocular thymic cysts, to exclude the possibility of neoplasm, especially when the cyst wall is thickened.

Primary solitary fibrous tumor of the retroperitoneum.

Solitary fibrous tumor is an uncommon neoplasm affecting adults and typically located in the pleura and can also occur in a large number of other extra thoracic sites. We present the case of a solitary fibrous tumor (SFT) of the retroperitoneum and describe their histopathological and immunohistochemical features. The identification of SFT in the retroperitoneum is of importance because its clinico-pathological behaviour is still unclear. The pathologist plays a fundamental role in establishing both the positive and differential diagnosis.

Spontaneous rupture of a recurrent hepatic cystadenoma.

Biliary cystadenoma is a rare cystic tumor of the middle aged woman that usually arises in the liver or occasionally in the extrahepatic bile ducts. It has a strong potential for recurrence and for malignant transformation. The lack of specific clinical and biological features hinders diagnosis before surgery. The spontaneous rupture of a hepatobiliary cystadnoma is a very rare and potentially life-threatening complication, with only two reported cases in the English literature. We report a case with spontaneous rupture of a recurrent hepatobiliary cystadenoma in a 32 year-old woman.