Calcifying fibrous tumour: An IgG4-related disease or not?

Calcifying fibrous tumour (CFT) has some of the histopathological features, such as abundant plasma cells and stromal fibrosis, that are exhibited by IgG4-related diseases (IgG4-RD). The possible role of IgG4-positive plasma cells in calcifying fibrous tumour was investigated. The aim of this study was to determine any potential relationship between IgG4-RD and CFT. Thirteen cases with a total of 16 CFTs were reviewed. Lesion samples were immunostained with anti-IgG4 and anti-IgG antibodies. The number of IgG4-positive and IgG-positive plasma cells (IgG + PC) and their ratios were estimated. Plasma cells were found in all tumours. IgG4-positive plasma cells ranged from 0 to 71 per high-power field (HPF; mean 17.8/HPF), and IgG + PC ranged from 2 to 93/HPF (mean 42.6/HPF). The IgG4/IgG ratio ranged from 0% to 80% (mean 29%). There were seven tumours with the ratio of IgG4/IgG + PC that exceeded 40%. Various degrees of stromal fibrosis were present in eight tumours. All tumours have variable calcification. The histopathological features of CFT were found to be similar to those of IgG4-RD. Some CFT also showed a high number of IgG4-positive plasma cells, and the ratio of IgG4/IgG + PC exceeded 40%, most notably in patients with concomitant inflammatory or autoimmune disease. The long-term follow-up showed no evidence of IgG4-RD in any of these patients. Our findings suggest that while CFT overlaps morphologically with IgG4-RD, it probably should not be classified as an IgG4-RD.

Recurrent gingival fibrous lesions: comparison of 2 cases and potential need for additional classification.

Historically, the classification of localized gingival fibrous lesions has been inconsistent, leading to multiple naming schemes and confusion among pathologists. Currently, lesions are broadly grouped into localized hyperplastic lesions and true neoplasms. Although some cases are clearly defined histologically (i.e., peripheral ossifying fibroma, peripheral odontogenic fibroma), another set of "reactive" fibrous lesions exhibit overlapping histologic features including nondistinctive fibrosis and inflammation. This group can exhibit recurrence, classically related to a local stimulus. However, when local factors are absent, recurrence suggests inherent neoplastic potential. Herein, we describe 2 recurrent fibrous gingival masses, one of which reportedly recurred 3 times with no obvious inciting agent. The histologic appearance of both lesions was similarly distinctive but not well classifiable, while the immunohistochemical profile indicated divergent lesions. This highlights the need for further study of recurrent gingival fibrous lesions to better predict independent growth potential.

[Fibroblastic-myofibroblastic proliferations of childhood and adolescents]. / Proliférations fibroblastiques et myofibroblastiques de l'enfance et de l'adolescence.

Fibroblastic-myofibroblastic proliferations of childhood and adolescents form a clinical and morphologic spectrum from benign reactive processes and pseudosarcomas, to fibromatoses, to various types of sarcoma. The diagnosis is challenging because of clinical and morphologic similarities, lack of specific immunohistochemical markers for different types of fibroblastic-myofibroblastic tumors, and limited molecular genetic information. Careful attention to clinical, macroscopic, and histopathologic features permits classification in most cases. This review focuses on the pathologic features of fibroblastic-myofibroblastic tumors with a predilection for children and adolescents.

The spectrum of pediatric fibroblastic and myofibroblastic tumors.

Fibroblastic and myofibroblastic tumors in neonates, infants, and children provide a diagnostic dilemma in surgical pathology due to their relative rarity and similarity in appearances. These tumors may be congenital or occur early during the first years of life or later during the first and second decades of life. The morphologic, immunocytochemical, ultrastructural, cytogenetic, and molecular features of the more "common" pediatric fibroblastic and myofibroblastic tumors are reviewed. In addition, the importance of a multimodal approach to tumor diagnosis is emphasized, with correlation with treatment and outcome differences among these unique fibroblastic and myofibroblastic tumors. The importance of providing an accurate diagnosis with pediatric fibroblastic and myofibroblastic tumors cannot be overstated, because treatment, prognosis, follow-up, and outcome are based on the initial assessment of these fascinating, but oftentimes, perplexing tumors.

Fibroblast phenotype plasticity: relevance for understanding heterogeneity in "fibroblastic" tumors.

Cellular transformations, reflecting phenotypic plasticity, characterize embryonic life, would-repair, physiological adaptation, and neoplasia. Fibroblastic tumors show a range of cellular differentiation, which can be rationalized in terms of phenotypic plasticity of the "normal" fibroblast. In this paper, the various kinds of fibroblast transformation are discussed, and some insights provided into the molecular mechanisms involved. Comparable molecular events may take place in neoplastic fibroblasts to produce the heterogeneous tumors nevertheless identified as fibroblastic. The following transformations are discussed: histiocytic, and fibrohistiocytic tumors; adipocytic, and lipogenic tumors; myofibroblastic, and myofibroblastic tumors. A definition of the fibroblast is required. This consists of spindle-cell morphology, vimentin-staining, and abundant rough endoplasmic reticulum. Transformation to histiocytic, lipogenic and myofibroblastic phenotypes requires the development of lysosomes, lipid droplets and lamina, and peripheral myofilaments and fibronexuses respectively. These occur in non-malignant transforming (transdifferentiating) fibroblasts, and also in tumors identified as fibrohistiocytic, lipogenic and myofibroblastic. The molecular basis of the myofibroblast transformation is probably the best studied. It is driven primarily by transforming growth factor beta. Investigations into the mechanisms of differentiation in normal fibrobiasts could prove fertile ground for defining comparable differentiation in tumors. In this respect, there are very few publications on the presence of growth factors in tumors or tumor-like lesions. There is, however, increasing investigation into gene expression and gene products in tumors, which bear on the differentiation process. Ultimately, our understanding of the molecular events controlling differentiation in cancer will lead to control, cure and prevention.

Metastatic malignant solitary fibrous tumour to the infra-temporal fossa.

Malignant solitary fibrous tumours (MSFTs) are rare tumours of fibrous origin, which can occur at all anatomical sites and represent 20% of solitary fibrous tumours. Fine-needle aspiration cytology is not able to distinguish benign from malignant disease, and sufficient tissue has to be obtained for accurate histological diagnosis to be made. Lesions > 10 cm in diameter and incomplete resection or non-resectability are predictive factors for poor long-term survival. We present a 57-year-old patient with a presumably metastatic MSFT from the peritoneal cavity to the skull-base who is in a stable state 17 months after surgical debulking of the skull-base and removal of the peritoneal lesion, followed by post-operative chemotherapy. We suggest the terminology metastatic malignant solitary fibrous tumour for a description of this disease.

Oral spindle cell neoplasms: a review of 307 cases.

The infrequent exposure of pathologists to soft tissue spindle cell neoplasms coupled with overlapping histologic patterns can often make diagnosis challenging. We reviewed all nonodontogenic spindle cell neoplasms seen between 1982 and 2002 (86,162 total accessions). Diagnoses were reclassified according to current standards supplemented with immunohistochemistry. Of the 307 neoplasms reviewed (0.36% of total accessions), neural tumors were the most common benign entities, accounting for 21% of total cases. Kaposi's sarcoma was the most common malignancy, accounting for 67% of all cases. Diagnoses were revised for 57 cases. Schwannoma and neurofibroma were most commonly revised to palisaded encapsulated neuroma. There were 8 myofibromas and 1 inflammatory myofibroblastic tumor. There were no oral leiomyomas; that is, all 4 originally reported cases were reclassified as myofibroma, palisaded encapsulated neuroma, and solitary fibrous tumor. With the exception of Kaposi's sarcoma, oral soft tissue sarcomas were rare; most benign lesions were neural in origin. The relatively high prevalence of some tumors, such as myofibroma, likely reflects the use of immunohistochemistry in the diagnosis of spindle cell tumors.

Imaging of benign and malignant soft tissue masses of the foot.

The foot is a relatively uncommon site of neoplastic and non-neoplastic soft tissue tumors. Although it contains a relatively small amount of somatic soft tissue elements, the foot is considerably rich in tendons, fasciae, retinaculae, and synovium. Corresponding to this distribution of soft tissue elements, some soft tissue lesions, such as giant cell tumor of tendon sheath, fibromatosis, and synovial sarcoma, are commonly seen in this location. Vascular tumors represent common soft tissue masses of the foot as well. Magnetic resonance imaging is the modality of choice in the assessment of soft tissue tumors. The presence of a suspected lesion can be confirmed and tumor margins can be defined accurately. In general, MRI does not provide histologic specificity, but considering some MR features may often help in correctly distinguishing benign from malignant lesions. In addition, characteristic features of the most common benign tumors (i.e., fibromatosis, cavernous hemangioma) and reactive processes of the foot (ganglion cyst, Morton's neuroma) often suggest a specific diagnosis.

Mesenchymal, non-meningothelial tumors of the central nervous system.

The spectrum of non-meningothelial mesenchymal tumors that may arise within the central nervous system is presented, based on the current classification of soft tissue tumors. Among malignant types, hemangiopericytoma, rhabdomyosarcoma, mesenchymal chondrosarcoma, and malignant fibrous histiocytoma are the most frequent ones. Rare tumor entities are mentioned. As in soft tissue sarcomas, diagnosis is mainly based on light and electron microscopy, while immunohistochemistry can improve accuracy of diagnosis.