Diagnostic difficulties of primary angiosarcoma of the breast: a case report.

BACKGROUND: Angiosarcoma of the breast is a rare tumor, which may be primary or secondary to breast surgery or irradiation. It is characterized by polymorphic and nonspecific clinical and radiological features. A pathologist plays a key role in positive and differential diagnosis and in establishing the prognosis: only a histological examination can confirm the diagnosis, and the histologic grade is the most important prognostic factor. In fact, angiosarcomas of the breast constitute a very heterogeneous group and they are classified into three grades based on the degree of differentiation. We will illustrate diagnostic challenges through this new case of primary angiosarcoma of the breast. Microscopic findings were initially interpreted as a benign vascular tumor. We will also discuss the relevant medical literature. CASE PRESENTATION: A 56-year-old Arabian woman presented with a palpable right breast mass that had been enlarging for 2 months, measuring 5 cm, without axillary lymphadenopathy. She had no personal or family history of breast surgery or breast irradiation. A mammography showed no evidence of spiculation. No suspicious calcifications were seen. A needle core biopsy was performed. Microscopic findings were initially interpreted as a benign vascular tumor. However, as the mass measured 5 cm, the diagnosis of angiosarcoma was more appropriate, and mastectomy without axillary dissection was performed. Microscopic examination found mild to moderately scattered pleomorphic cells, and scattered mitotic figures. It also showed papillary formations, solid foci of spindle cells, and hemorrhagic necrosis. The margins of the tumor were infiltrative. The diagnosis of primary intermediately differentiated angiosarcoma of the breast (grade II) was made. No distant metastases were found. Our patient was lost to follow-up and further treatment after mastectomy until she developed local tumor progression 4 months later. CONCLUSIONS: Through this case report, we emphasize the importance of clinicopathological confrontation in angiosarcoma of the breast.

Intracardiac Teratoma in an Infant: Report of a New Case and Literature Review.

Primitive intracardiac tumours are rare, especially in childhood, and are often discovered on autopsy. The intracardiac teratoma is the rarest intracardiac tumours of childhood. Herein, we report the case of an 11-month-old infant, which featured recurrent bronchoalveolitis since the age of 3 months, with a thoracic deformation. Physical examination did found discrete respiratory distress signs. Chest radiography showed large mediastinal enlargement. The computed tomography showed a solid cystic-cloisonned mass with fat and central calcification highly suggestive of an intracardiac teratoma. A radical surgical excision was made and the histological examination found a well circumscribed tumour containing elements of the three germ layers confirming the diagnosis of mature well-differentiated teratoma, with no need of immunohistochemical support.

[Unusual giant cell fibroblastoma: A case report]. / Fibroblastome à cellules géantes inhabituel: à propos d'un cas.

Giant cell fibroblastoma (GCF) is a rare superficial tumor occurring in male infants. We report the case of a 3-month old infant examined for a mass in his right arm. Arm MRI objectified a subcutaneous mass. The patient underwent total excision of the mass. Histological, immuno-histochemical and genetic examinations showed giant cell fibroblastoma. This tumor poses diagnostic challenges to the pathologist because it may be confused with malignant mesenchymal tumors with different prognosis.

Peripheral primitive neuroectodermal tumors of the spine: a case report and review of the literature.

BACKGROUND: Peripheral primitive neuroectodermal tumors are extremely rare tumors in the spine; only 18 cases of extra-dural peripheral primitive neuroectodermal tumor cervical region have been reported. The aim of this report is to highlight the challenges in diagnosis and management of this condition. CASE PRESENTATION: We present a case of 5-year-old Moroccan boy, who presented with torticollis for 1 month. Computed tomography scan and Magnetic resonance imaging of the cervical spine revealed an extradural, dumbbell-shaped mass with extra-spinal extension at the left C1-C6 level. Multiple biopsy specimens were obtained. Histological examination revealed a highly cellular neoplasm composed of diffuse sheets of tumor cells having monomorphic, round to oval, finely vesicular nuclei. Immunohistochemical findings confirmed the diagnosis of intraspinal peripheral primitive neuroectodermal tumor. CONCLUSION: After this illustrative case, we review the literature on clinicopathological and therapeutic aspects. In practice, it is important to consider the diagnosis of peripheral primitive neuroectodermal tumor in children and adolescents with an apparent soft-tissue mass located in the spine.

Cellular angiofibroma of the vulva: a poorly known entity, a case report and literature review.

BACKGROUND: Cellular angiofibroma represents a newly described, site specific tumor. Histologically, CAF is a benign mesenchymal neoplasm characterized by two principal components: bland spindle cells and prominent small to medium-sized vessels with mural hyalinization. The indolent nature of the lesion is underscored by the uniformity of its constituent stromal cells, and their lack of nuclear atypia. Characterization by immunohistochemistry is helpful distinguishing Cellular angiofibroma from other mesenchymal lesions. CASE PRESENTATION: We report the case of a 37-year-old woman, presenting with a painless nodule involving the vulva. This lesion had gradually increased in size; a simple excision was performed, and follow up was unremarkable. Gross examination showed a well circumscribed, firm tumor measuring 3× 3 × 2,5 cm. Histologically, the tumor was composed of uniform, short spindle-shaped cells, proliferating in an edematous to fibrous stroma and numerous small to medium-sized thick-walled vessels. A panel of immunohistochemical stains was performed, and confirmed the diagnosis of Cellular angiofibroma. CONCLUSION: In this report we aim to describe the clinical, pathological and immunohistochemical features of this rare entity through a literature review, and to discuss other vulvar mesenchymal lesions.

[Pathological and clinical correlations in renal AA amyloidosis: A Moroccan series of 30 cases]. / Amyloses rénales AA : corrélations anatomocliniques dans une série marocaine de 30 cas.

OBJECTIVE: Study of histological and clinical correlations of 30 cases of renal amyloidosis AA diagnosed between November 2010 and December 2012. RESULTS: The main causes associated with amyloidosis AA were represented by chronic infectious diseases (60%). Nephrotic syndrome and renal failure were observed in 94% and 73% respectively. The distribution of amyloid deposits: 90% of patients had a glomerular form and 10% had a vascular form. Inflammatory reaction associated with AA renal amyloidosis was present in 50% of cases. This inflammation was observed near amyloid deposits associated with a deposition of immunoglobulin chains and/or complement factors. CONCLUSION: Our study confirms the predominance of AA amyloidosis complicating chronic infectious diseases, especially tuberculosis. Our data point out a relationship between the morphology of renal AA amyloidosis, its clinical presentation and prognosis.