A case of intravascular large B cell lymphoma: New clinical and immunohistochemical findings.

Intravascular large B cell lymphoma (IVLBCL) is a rare extranodal non-Hodgkin lymphoma characterized by proliferation of malignant cells within the lumen of small vessels, with a predilection for the CNS and the skin. IVLBCL clinical course is highly aggressive, clinical signs and symptoms are not specific and may consist of neurological and cognitive impairment, fever of unknown origin and cutaneous lesions, lacking of a typical neuroimaging pattern. For all these reasons the diagnosis is commonly missed and the exitus is frequent, therefore post mortem evaluation is necessary to clarify the clinical history. We present a case of IVLBCL in a 62-year-old woman with unusual symptomatology, mimicking a vascular, multi-infarctual cerebropathy. Hachinski Ischemic Score was 7 suggesting a vascular dementia. Autopsy was unable to define the nature of the disease. Immunohistochemical analysis for cluster of differentiation 20 (CD20) revealed the ubiquitous presence of malignant lymphoid B-cells into the vessel of all organs analyzed, allowing the definitive diagnosis of IVLBCL. The atypical cells expressed high levels of anti-apoptotic proteins B-cell lymphoma 2 (Bcl-2) and Galectin-3, and showed cellular myelocytomatosis (c-Myc) staining in <50% of tumor nuclei. Conversely, cells were immunonegative for multiple myeloma-1 (MUM1), CD3, CD44, CD30, CD34 and CD133. Fluorescent in situ hybridization analysis for MYC rearrangements was negative. The high expression of Galectin-3 provides new insights in the understanding of molecular pathogenesis of IVLBCL; indeed, such a finding represents a prognostic factor for other types of lymphoma and should, in the same way, be taken into account in IVLBCL.

MRI findings of a remote and isolated vaginal metastasis revealing an adenocarcinoma of the mid-sigmoid colon.

A remote vaginal metastasis from a colo-rectal carcinoma is extremely rare. Only few cases have been described in the literature. The radiological appearances of a vaginal metastasis from colon-rectal cancer have not been extensively investigated. We report the MRI findings with clinical and pathological correlations of a remote and isolated vaginal metastasis revealing a mid-sigmoid adenocarcinoma in a 67 years old woman.

Radiofrequency-assisted partial nephrectomy for metanephric adenoma: a case report and literature review.

Metanephric adenoma (MA) is a rare benign tumor, diagnosis of which is often carried out after surgical treatment. In case of peripheral lesions, a partial nephrectomy (PN)--either open or laparoscopic may be preferred--and, furthermore, a radiofrequency (RF)-assisted procedure may facilitate adequate hemostasis. In November 2010, the authors performed a RF-assisted PN, according to Habib's technique, using a 4-needle bipolar device, on a woman affected by a small exophytic MA of the right kidney. Fibrin glue was applied on the cut surface. Postoperative course was uneventful, and discharge was on postoperative day 4. MA is an extremely rare benign tumor with a favorable prognosis. In case of a preoperative cytological diagnosis, a careful follow-up has to be considered. PN represents the standard of care for small exophytic MA, and RF-assisted procedures allow an excellent hemostasis and a rapid conservative resection, with very low morbidity.

Expression Profile of Stemness Markers CD138, Nestin and Alpha-SMA in Ameloblastic Tumours.

Ameloblastic carcinoma is a rare malignant odontogenic neoplasm with a poor prognosis. It can arise de novo or from a pre-existing ameloblastoma. Research into stemness marker expression in ameloblastic tumours is lacking. This study aimed to explore the immunohistochemical expression of stemness markers nestin, CD138, and alpha-smooth muscle actin (alpha-SMA) for the characterisation of ameloblastic tumours. Six cases of ameloblastoma and four cases of ameloblastic carcinoma were assessed, including one case of ameloblastic carcinoma arising from desmoplastic ameloblastoma. In all tumour samples, CD138 was positive, whilst alpha-SMA was negative. Nestin was negative in all but one tumour sample. Conversely, the presence or absence of these markers varied in stroma samples. Nestin was observed in one ameloblastic carcinoma stroma sample, whilst CD138 was positive in one ameloblastoma case, one desmoplastic ameloblastoma case, and in two ameloblastic carcinoma stroma samples. Finally, alpha-SMA was found positive only in the desmoplastic ameloblastoma stroma sample. Our results suggest nestin expression to be an indicator for ameloblastic carcinoma, and CD138 and alpha-SMA to be promising biomarkers for the malignant transformation of ameloblastoma. Our data showed that nestin, CD138, and alpha-SMA are novel biomarkers for a better understanding of the origins and behaviour of ameloblastic tumours.

Primary peritoneal clear cell carcinoma versus ovarian carcinoma versus malignant transformation of endometriosis: a vexing issue.

Peritoneum is a site for both primary and secondary tumors. Primary peritoneal tumors are fairly rare. The most common primary tumors of the peritoneum are malignant mesothelioma and serous papillary adenocarcinoma. Clear cell carcinoma of the peritoneum is extremely rare and often misdiagnosed as mesothelioma, serous carcinoma, or metastatic adenocarcinoma, so it represents a diagnostic challenge for both clinicians and pathologists. Up to date, to the best of our knowledge, only 11 cases of primary peritoneal clear cell carcinoma have been reported in the English literature. Distinguishing this tumor of the peritoneum versus ovarian carcinoma can be problematic. Herein, we report a rare case of primary peritoneal clear cell carcinoma occurring in a 49-year-old woman, along with a review of the literature.

A prospective randomized study on limits of colposcopy and histology: the skill of colposcopist and colposcopy-guided biopsy in diagnosis of cervical intraepithelial lesions.

BACKGROUND: The main objective of our study was to evaluate the colposcopist ability to correctly identify the worst area of a cervical lesion where biopsy should be performed; the secondary objective was to investigate the influence of the colposcopist skill in grading cervical preneoplastic lesions. METHODS: 296 patients referred for colposcopy were enrolled in a prospective study. All patients were randomized in two groups: in the first group, "senior group", the colposcopy was performed by an experienced colposcopist; in the second group, "junior group", the colposcopy was performed by a less experienced colposcopist. A detailed colposcopic description, including a grading of the lesion, was completed for each case. During the colposcopic exam patients underwent two direct biopsies; each biopsy was labeled with letter A (suspicious area with most severe grade) or B (suspicious area with less severe grade) according to the judgment of the colposcopist. An experienced pathologist reanalyzed the histological slides, after routine diagnosis. RESULTS: The senior group identify the worst area of the cervical lesion in statistical significant higher rates than junior group. Specimen A resulted representative of the higher-grade lesion (A > B) in 73.7 % (N = 28) in senior group and in 48.4 % (N = 15) in junior group; while in 26.3 % (N = 10) the higher-grade lesion corresponded to specimen B (A < B) in senior group and in 51.6 % (N = 16) in junior group (p < .05). CONCLUSION: The ability of a colposcopist in grading cervical lesion depends on his experience.

An unusual association of malignant gastrointestinal neuroectodermal tumor (clear cell sarcoma-like) and Ewing sarcoma.

Very recently a new designation of "Malignant Neuroectodermal Gastrointestinal Tumor" has been proposed for an aggressive form of neuroectodermal tumor with features similar to that of Clear Cell Sarcoma of Soft Tissue, however without a melanocytic differentiation. Also known as "clear cell sarcoma-like tumors of the gastrointestinal tract", these tumors show some features strongly suggesting an origin from a gastrointestinal neuroectodermal precursor cell unable to differentiate along the melanocytic lineage. They occur mainly in young and middle-aged adults, and have a poor prognosis with a high rate of liver and lymphnode metastases. Histologically they are composed of epithelioid or oval-to spindle cells with a sheet-like or nested pattern of growth, strongly positive for neural markers (S-100, SOX10, and vimentin) and negative for the melanocytic ones. EWSR1 gene rearrangements including EWSR1-ATF1 or EWSR1-CREB1 GENE fusions are typically assessed in these tumors. Here we report a case of malignant neuroectodermal gastrointestinal tumor which immunophenotypically unusually expressed FLI-1, occurring in a 29-year-old man with a previous medical history of Ewing sarcoma. We finally suggest that this case might be a further evidence of a link between these two entities.

Metastasis to the kidney from a B2 thymoma: report of a case.

Thymomas are tumors with an indolent behavior and long clinical course, usually confined to the anterior mediastinum. Local recurrence is frequent while distant metastases are extremely rare. We report an unusual case of a B2 thymoma with a single renal metastasis The challenge in this case was the frozen section intraoperative diagnosis because of the clinical suspicion of malignant lymphoma. This report also highlights the role of surgical removal of solitary metastases.

A huge malignant perivascular epithelioid cell tumor (PEComa) of the uterine cervix and vagina.

Perivascular epithelioid cell tumors (PEComas) are a family of rare mesenchymal neoplasms, including angiomyolipoma, clear-cell "sugar" tumor of the lung and extrapulmonary sites, lymphangioleiomyomatosis, clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and clear-cell tumors at various other anatomic sites. These tumors are characterized by a proliferation of epithelioid cells with clear to eosinophilic cytoplasm, perivascular distribution, and coexpression of myogenic and melanocytic markers. PEComas show a female predominance, occur with some frequency in the gynecological tract, and have an unpredictable biological behavior. We report a case of a huge malignant PEComa arising from the uterine cervix and vagina. To the best of our knowledge, only 6 cases of PEComa in the cervix and 2 cases in the vagina have been reported in the literature.

Lymph nodes fine needle cytology in the diagnosis of infectious diseases: clinical settings.

Lymph node reactive hyperplasia, caused by specific infectious etiologic factors, represents the most frequent cause of enlarged peripheral lymph nodes. The main infectious agents are viruses, pyogenic bacteria, mycobacteria, fungi and protozoa that may determine unspecific or specific pathological entities, such as cat-scratch disease, toxoplasmosis or infectious mononucleosis. Lymph node fine needle cytology (FNC) is a safe, simple, cost-effective and efficient technique that quickly provides information about the cell population and the nature of the process. FNC can also provide suitable material for ancillary techniques, such as flow cytometry, immunocytochemistry, molecular biology and microbiological examinations. This study focuses on the cytological features of benign lymphadenopathy of infectious origin and their possible contribution to the clinical setting definition of corresponding patients.

Mast cell sarcoma of the scalp: the first sign of undisclosed systemic mastocytosis?

Mastocytosis is a neoplastic disease of mast cells and their CD34+ precursors, including a heterogeneous group of disorders. It is characterized by abnormal growth and accumulation of mast cells in one or more organ systems. Mast cell sarcoma is an extremely rare and aggressive disease characterized by local proliferation of atypical mast cells, destructive growth and poor prognosis, without systemic involvement. Very few clinical cases describing this entity have been reported in the literature. In this paper, we report a case of a mast cell sarcoma, localized in the scalp of a 63-year-old woman; it appears to be the first manifestation of undisclosed systemic mastocytosis.

Symplastic glomus tumor: report of a challenging lesion with literature review.

Glomus tumors are uncommon mesenchymal tumors whose cells closely resemble those of a normal glomus body, and are found most commonly in the hand. Recently, a symplastic form exhibiting marked nuclear atypia, in the absence of any other malignant features, has been described. To date, only 14 cases of symplastic glomus tumor have been reported in the literature; hence, very little information is available about its diagnosis, treatment, and biological behavior. The case reported here concerns a symplastic glomus tumor occurring in the right index finger of a 62-year-old woman. We reviewed the literature concerning previously reported cases, placing emphasis on the documented biological behavior, treatment, and demographic trend. Physicians must be aware of this morphological variation of glomus tumor to avoid the mistake of malignancy, which results in over-treatment of the patient.