Uterine inflammatory myofibroblastic tumors in pregnant women with and without involvement of the placenta: a study of 6 cases with identification of a novel TIMP3-RET fusion.

Uterine inflammatory myofibroblastic tumors (IMTs) have been reported in association with pregnancy and, in some instances, secondarily involve the placenta. The clinicopathological spectrum of these tumors in the setting of pregnancy is not well defined. We investigated the clinical, morphologic, immunohistochemical, molecular cytogenetic, and genetic features of 6 uterine IMTs occurring in pregnant women. Each tumor was discovered at parturition, and none was identified by prenatal ultrasound. Patient age ranged from 25 to 41 years (mean 31.5). Tumor size ranged from 1.5 to 9 cm (mean 4.7). Four of 6 had usual IMT features, with at least focal deciduoid change in 3. Necrosis was identified in 3 tumors; and multinucleated cells, in 3 tumors. Sex hormone receptor expression was consistent with estrogen receptor negative or focally weakly positive and progesterone receptor diffusely moderately or moderately to strongly positive in all 6 tumors. ALK immunohistochemistry was strongly positive in 5 tumors, and all of these had an ALK rearrangement detected by break-apart fluorescence in situ hybridization. Subsequent RNA sequencing of these 5 tumors identified a TIMP3-ALK fusion in 4 and a THBS1-ALK in 1. In the ALK-negative tumor, RNA sequencing detected a novel TIMP3-RET fusion that was confirmed by RET break-apart fluorescence in situ hybridization. Follow-up was available for 2 of 6 patients 5 and 19 months after diagnosis. Neither patient developed recurrence. ALK immunohistochemistry will distinguish most uterine IMTs, but if ALK expression and gene studies are negative, in the appropriate morphologic context, evaluation of other tyrosine kinase genes known to be more commonly altered in extrauterine IMTs such as ROS1, NTRK3, PDGFRß, and RET may be necessary for diagnostic confirmation.

Calcifying Fibrous Tumor of the Tunica Vaginalis Testis: A Report of 2 Cases.

OBJECTIVE: To improve the clinical diagnosis and treatment of calcifying fibrous tumor (CFT) of the tunica vaginalis testis, we discussed clinical manifestations and pathologic features of CFT. MATERIALS AND METHODS: A retrospective analysis of 2 cases of CFT that occurred in our hospital was performed, and we also reviewed the literature and research reports. RESULTS: Both patients underwent local excision with testis preservation surgery, and pathology examination confirmed the presence of multiple CFTs of the tunica vaginalis testis. CONCLUSION: CFTs are rare, benign multiple lesions, and the recognition of which needs to be improved to avoid overtreatment. We are the first to describe 2 cases of CFT combined with hepatitis B, but additional studies are needed to confirm the relationship between these conditions.

Tumor fibroso solitario de cavidad oral. Confirmación histológica final ante la sospecha de lipoma facial / Solitary fibrous tumor of the oral cavity. Final histologic confirmation after suspected facial lipoma

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A biphasic tumor consisting of pilocytic astrocytoma with an anaplastic solitary fibrous tumor component in the pineal region: a case report and literature review.

Here we report a case of a biphasic tumor consisting of pilocytic astrocytoma with anaplastic solitary fibrous tumor component in the pineal region. The majority of the tumor showed typical histologic features of pilocytic astrocytoma. A minor part of the tumor showed marked proliferation of short spindle cells around vessels. These spindle cells showed CD34 and CD99 immunoreactivity. From a review of the literature, we found that only one similar case has been reported. Contrary to the reported case, our case showed anaplastic features of solitary fibrous tumor histology.

Development of a novel gene silencer pyrrole-imidazole polyamide targeting human connective tissue growth factor.

Pyrrole-imidazole (PI) polyamide can bind to specific sequences in the minor groove of double-helical DNA and inhibit transcription of the genes. We designed and synthesized a PI polyamide to target the human connective tissue growth factor (hCTGF) promoter region adjacent to the Smads binding site. Among coupling activators that yield PI polyamides, 1-[bis(dimethylamino)methylene]-5-chloro-1H-benzotriazolium 3-oxide hexafluorophosphate (HCTU) was most effective in total yields of PI polyamides. A gel shift assay showed that a PI polyamide designed specifically for hCTGF (PI polyamide to hCTGF) bound the appropriate double-stranded oligonucleotide. A fluorescein isothiocyanate (FITC)-conjugated PI polyamide to CTGF permeated cell membranes and accumulated in the nuclei of cultured human mesangial cells (HMCs) and remained there for 48 h. The PI polyamide to hCTGF significantly decreased phorbol 12-myristate acetate (PMA)- or transforming growth factor-ß1 (TGF-ß1)-stimulated luciferase activity of the hCTGF promoter in cultured HMCs. The PI polyamide to hCTGF significantly decreased PMA- or TGF-ß1-stimulated expression of hCTGF mRNA in a dose-dependent manner. The PI polyamide to hCTGF significantly decreased PMA- or TGF-ß1-stimulated levels of hCTGF protein in HMCs. These results indicate that the developed synthetic PI polyamide to hCTGF could be a novel gene silencer for fibrotic diseases.

Solitary fibrous tumor of the lung: a case report with a study of the aspiration biopsy, histopathology, immunohistochemistry, and autopsy findings.

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm of ubiquitous location. In addition to its classic presentation as a pleural-based mass, it can be encountered in a variety of other sites. A pleural-based lung lesion can be easily accessed by radiologic guidance for cytologic study. Several reports have described the cytologic findings of SFT at various locations, including the lung. However, diagnostic difficulties can occur due to unusual clinical, radiologic, atypical cytomorphologic, and histologic features. We describe a case of intrapulmonary SFT in which a false-positive malignant diagnosis was rendered on fine-needle aspiration biopsy and concurrent surgical core biopsy prior to radiofrequency ablation. The patient died of procedural complications, and an autopsy was performed. Retrospective study of the case, especially correlation of cytologic, histologic, autopsy findings, and immunohistochemistry results were helpful in correctly diagnosing the case as SFT. We are reporting this case with emphasis on avoiding diagnostic pitfalls by being familiar with the accepted cytohistologic features and appropriate immunohistochemical results.

Solitary fibrous tumor of the pleura.

BACKGROUND: The solitary fibrous tumor of the pleura (SFTP) is a rare primary tumor arising from mesenchymal cells in the areolar tissue subjacent to the mesothelial-lined pleura. Only about 800 cases have been reported in the medical literature. The tumor appears to be unrelated to malignant pleural mesothelioma, the most common primary tumor of the pleura. METHODS: In just over half of these cases, the neoplasm presents as an asymptomatic mass, is often quite large, and is benign in 78% to 88% of patients. The initial evaluation and diagnosis, tumor classification, surgical treatment, results of therapy, and long-term prognosis are reviewed, based on a selective review of the literature from MEDLINE beginning 1980. RESULTS: Complete en bloc surgical resection is the preferred treatment of benign and malignant varieties of the tumor. The pedunculated tumors attached to the visceral pleura can be effectively treated with a wedge resection of lung. Sessile tumors arising on the lung require a larger lung resection. Sessile tumors on the chest wall require wide local excision, often with chest wall resection because of their propensity for local recurrence. Adjuvant therapy remains controversial in SFTP. CONCLUSIONS: Benign SFTP has a high cure rate and an 8% local recurrence rate that is usually amenable to curative re-excision. Malignant SFTP, especially the more common sessile type, has a 63% recurrence rate even with complete resection. The majority of patients with recurrent disease die of the tumor within 2 years. Nevertheless, the overall long-term cure rate for all patients is 88% to 92%.

Solitary fibrous tumour of the pleura.

Solitary fibrous tumours of the pleura are rare tumours originating from the mesenchymal cells of the submesothelial tissue of the pleura. The tumours may present in a variety of ways, ranging from no symptoms, to local symptoms such as dyspnoea, cough and chest pain, through to systemic symptoms such as clubbing and hypoglycaemia. We present a case of a solitary fibrous tumour, which presented with clubbing.

A highly vascular intracranial solitary fibrous tumor treated with radiotherapy and toremifene: case report.

OBJECTIVE AND IMPORTANCE: A case of an unusual intracranial solitary fibrous tumor with features of high vascularity and resultant difficulty at resection is presented. The use of surgery, radiotherapy, and toremifene has been successful with no recurrence after 18 months. CLINICAL PRESENTATION: A 33-year-old male patient presented with a 1-year history of loss of vision on the left side and a 3-month history of headache and lethargy. Magnetic resonance imaging showed a lobulated mass (7.6 x 4.5 cm) in the left temporal fossa and left parasellar, sellar, and suprasellar regions, with a "dural tail" more typical of meningioma. Vascular supply was from both the internal carotid arteries and the left middle meningeal artery, precluding embolization. INTERVENTION: At the time of the craniotomy, a highly vascular tumor was found. Intraoperative hemorrhage limited resection to 20% of the tumor. The histological diagnosis was of a solitary fibrous tumor with an unusually angiomatoid architecture. After the operation, radiotherapy and toremifene were administered. A dramatic reduction in the size of the tumor was seen with no recurrence to date. CONCLUSION: The use of surgery, radiotherapy, and toremifene in treatment of a vascular intracranial solitary fibrous tumor has been successful and warrants further research.

Preoperative embolization of a massive solitary fibrous tumor of the pleura.

We report the case of a 69-year-old woman with a massive fibrous tumor of the left hemithorax. Initial attempted removal by median sternotomy was abandoned due to the extremely vascular appearance of the tumor and the inability to gain safe control of the pedicle. Subsequent percutaneous embolization before removal through a left thoracotomy was successful, with little intraoperative blood loss. We recommend preoperative angiography for massive chest tumors that may be vascular and require piecemeal removal for total excision.

June 2001: 61 year old woman with confusion and obtundation.

The June COM. A 61 year old female presents with a three week history of increasing confusion, lethargy and headache. A neurological exam revealed disorientation, mild expressive aphasia, bilateral papilledema, and a right pronator drift. She had a craniotomy and resection of tumor. The tumor histologically was consistent with a solitary fibrous tumor displaying malignant features of hypercellularity, marked nuclear atypia, high mitotic activity, and a high proliferation index. This case is unique as the first malignant variant of solitary fibrous tumor to be reported intracranially.

Fibrous tumors of the pleura.

Fibrous tumor of the pleura is a rare tumor arising from mesenchymal cells underlying the visceral or parietal pleura. The tumor may have benign or malignant histological features, but these do not always predict the clinical behavior of the tumor. In most cases, the tumor appears pedunculated, and simple resection of the tumor is curative even if significant cellular atypia is present. In contrast, some tumors with a broad base of attachment may recur and occasionally become malignant. Complete surgical resection is the mainstay of therapy for both benign and malignant fibrous tumors of the pleura. When resection is incomplete or impossible, external radiation therapy with or without chemotherapy is recommended.

Malignant solitary fibrous tumor arising in the right buttock associated with metastatic parietal pleural and intrapulmonary tumors in addition to pleural effusion.

A malignant solitary fibrous tumor arising in the right buttock associated with metastatic parietal pleural and intrapulmonary tumors and pleural effusion was found in a 59-year-old man. A chest computed tomogram revealed three tumors attached to the parietal pleura with rib destruction, and a tumor in the left lower lung field. Histologically, the tumors of the buttock and parietal pleura were characterized by proliferation of bundles of spindle-shaped or oval cells separated by wavy hyalinized collagen tissue with no expression of cytokeratin, S-100 protein, muscle actin or epithelial membrane antigen, but these cells weakly expressed CD34 and strongly expressed vimentin.

[Tumors of the mesentery. Apropos of 102 cases]. / Les tumeurs du mésentère. A propos de 102 cas.

One hundred and two mesenteric tumours or masses are reported, and their histologic, clinical and therapeutic aspects are described: 28 cystic tumours, 38 mesenchymatous and 3 neurogenic tumours, 27 miscellaneous tumours, including 17 lymphomas, 2 hematomas and lymphoid or inflammatory masses. Seventy-four patients presented with an abdominal mass, 41 with pain and 28 with acute abdominal syndrome, including 16 with intestinal obstruction. Ultrasonography and computerised tomography were performed in 31 patients, RMI in 8 patients: these investigations are useful in establishing the diagnosis but surgical exploration and biopsies remain necessary for the pathology. One hundred and one patients required operation: 45 biopsies with 5 by-passes, and 56 resections, 32 localized tumour resections and 24 extended to the small bowel. Radiotherapy and chemotherapy have little or no effect.