MR imaging features and a redefinition of the classification system for nodular fasciitis.

To analyze magnetic resonance imaging features of nodular fasciitis and redefine the system for classifying this class of lesions.Twenty-seven patients with nodular fasciitis and 71 patients with other soft tissue lesions who underwent surgery or biopsy were retrospectively analysed. Demographic information, medical history, and magnetic resonance imaging features were collected. Classification of nodular fasciitis was performed based on a redefined system. Comparison between 2 groups was performed with Chi-square or Fisher exact test.For nodular fasciitis, the longest average lesion diameter was 1.87 cm (range, 0.52-5.46 cm), and 40.7% of lesions were located in the upper extremities, while 29.6% were located in the head and neck. Compared with skeletal muscle, most lesions exhibited isointensity on T1-weighted imaging and hyperintensity on T2-weighted imaging, and 45.5% of the lesions exhibited rim enhancement, 40.9% showed obvious homogenous enhancement, while 13.6% showed no enhancement or slight enhancement. The subcutaneous type accounted for 25.9% of cases, the fascial type 25.9%, the intramuscular type 29.6%, and the unclassified type 18.5%. The "fascia tail sign" was more frequently observed in nodular fasciitis than in other soft tissue lesions (P < .001). Nodular fasciitis was slightly more likely to present with the "inverted target sign" and "solar halo sign" than other soft tissue lesions (P > .05). The "cloud sign" only appeared in nodular fasciitis (P < .05).The "fascia tail sign" and "cloud sign" could help differentiate nodular fasciitis from other soft tissue lesions. A new classification may improve understanding about nodular fasciitis.

The EGS grading scale for skin and soft-tissue infections is predictive of poor outcomes: a multicenter validation study.

INTRODUCTION: Over the last 5 years, the American Association for the Surgery of Trauma has developed grading scales for emergency general surgery (EGS) diseases. In a previous validation study using diverticulitis, the grading scales were predictive of complications and length of stay. As EGS encompasses diverse diseases, the purpose of this study was to validate the grading scale concept against a different disease process with a higher associated mortality. We hypothesized that the grading scale would be predictive of complications, length of stay, and mortality in skin and soft-tissue infections (STIs). METHODS: This multi-institutional trial encompassed 12 centers. Data collected included demographic variables, disease characteristics, and outcomes such as mortality, overall complications, and hospital and ICU length of stay. The EGS scale for STI was used to grade each infection and two surgeons graded each case to evaluate inter-rater reliability. RESULTS: 1170 patients were included in this study. Inter-rater reliability was moderate (kappa coefficient 0.472-0.642, with 64-76% agreement). Higher grades (IV and V) corresponded to significantly higher Laboratory Risk Indicator for Necrotizing Fasciitis scores when compared with lower EGS grades. Patients with grade IV and V STI had significantly increased odds of all complications, as well as ICU and overall length of stay. These associations remained significant in logistic regression controlling for age, gender, comorbidities, mental status, and hospital-level volume. Grade V disease was significantly associated with mortality as well. CONCLUSION: This validation effort demonstrates that grade IV and V STI are significantly predictive of complications, hospital length of stay, and mortality. Though predictive ability does not improve linearly with STI grade, this is consistent with the clinical disease process in which lower grades represent cellulitis and abscess and higher grades are invasive infections. This second validation study confirms the EGS grading scale as predictive, and easily used, in disparate disease processes. LEVEL OF EVIDENCE: Prognostic/Epidemiologic retrospective multicenter trial, level III.

Actualización en la clasificación y el tratamiento de la esclerodermia localizada / Update on the Classification and Treatment of Localized Scleroderma

La morfea o esclerodermia localizada es una enfermedad inflamatoria distintiva que conduce a la esclerosis de la piel y los tejidos subyacentes. Incluye una serie de entidades que pueden distinguirse basándose en las manifestaciones clínicas y la estructura de la piel y los tejidos subyacentes involucrados en el proceso fibroso. Sin embargo, la clasificación de estos procesos resulta difícil desde el momento en que los límites entre ellos no siempre son claros y es frecuente el solapamiento. En esencia, se distingue entre la morfea en placas, la esclerodermia lineal, la morfea generalizada y la panesclerótica. Si bien no tiene, salvo excepciones, una repercusión sistémica grave, sí que puede ser causa de una gran morbilidad. Si las lesiones asientan en el polo cefálico, pueden acompañarse de complicaciones neurológicas y oculares. No existe un tratamiento realmente eficaz y universal por lo que es importante realizar una evaluación correcta de la extensión y la gravedad de la enfermedad antes de tomar una decisión terapéutica (AU)

Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. However, classification is difficult because the boundaries between the different types of morphea are blurred and different entities frequently overlap. The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea. With certain exceptions, the disorder does not have serious systemic repercussions, but it can cause considerable morbidity. In the case of lesions affecting the head, neurological and ocular complications may occur. There is no really effective and universal treatment so it is important to make a correct assessment of the extent and severity of the disease before deciding on a treatment approach (AU)

Update on the classification and treatment of localized scleroderma.

Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. However, classification is difficult because the boundaries between the different types of morphea are blurred and different entities frequently overlap. The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea. With certain exceptions, the disorder does not have serious systemic repercussions, but it can cause considerable morbidity. In the case of lesions affecting the head, neurological and ocular complications may occur. There is no really effective and universal treatment so it is important to make a correct assessment of the extent and severity of the disease before deciding on a treatment approach.

Myoepithelial and epithelial-myoepithelial, mesenchymal and fibroepithelial breast lesions: updates from the WHO Classification of Tumours of the Breast 2012.

In the 4th edition of the WHO Classification of Tumours of the Breast, myoepithelial lesions are retitled myoepithelial and epithelial-myoepithelial lesions in order to better reflect the dual participation of luminal and myoepithelial compartments in some key entities. Malignant myoepithelioma, described as a section within the chapter on myoepithelial lesions in the 3rd edition, is recognised in the 4th edition as part of metaplastic carcinoma. Adenomyoepithelioma with malignancy is categorised in terms of the cellular component undergoing malignant transformation. The list of antibodies that can be used for identifying myoepithelial cells is updated. Among mesenchymal lesions, new additions are nodular fasciitis and atypical vascular lesions, while the haemangiopericytoma is removed. The 3rd edition stated that pathological prediction of behaviour of phyllodes tumours is difficult in the individual case. In the 4th edition, some progress has been made in prioritisation and weighting of histological parameters that can potentially estimate probability of recurrence. The WHO Working Group advocates leaning towards a diagnosis of fibroadenoma in cases where there is histological uncertainty in distinction from a benign phyllodes tumour, or adopting the neutral term 'benign fibroepithelial neoplasm', as the clinical behaviour of fibroadenoma overlaps with that of benign phyllodes tumour. The 3rd edition terminology of 'periductal stromal sarcoma' is revised to 'periductal stromal tumour', akin to the widespread consensus to avoid the use of the term 'cystosarcoma' in the context of phyllodes tumours.

Nodular fasciitis with degeneration and regression.

Nodular fasciitis is a benign reactive proliferation that is frequently misdiagnosed as a sarcoma. This article describes a case of nodular fasciitis of 6-month duration located in the cheek, which degenerated and spontaneously regressed after biopsy. The nodule was fixed to the zygoma but was free from the overlying skin. The mass was 3.0 cm in diameter and demonstrated high signal intensity on T2-weighted magnetic resonance imaging. A small part of the lesion was biopsied. Pathological and immunohistochemical examinations identified the nodule as nodular fasciitis with myxoid histology. One month after the biopsy, the mass showed decreased signal intensity on T2-weighted images and measured 2.2 cm in size. The signal on T2-weighted images showed time-dependent decreases, and the mass continued to reduce in size throughout the follow-up period. The lesion presented as hypointense to the surrounding muscles on T2-weighted images and was 0.4 cm in size at 2 years of follow-up. This case demonstrates that nodular fasciitis with myxoid histology can change to that with fibrous appearance gradually with time, thus bringing about spontaneous regression. Degeneration may be involved in the spontaneous regression of nodular fasciitis with myxoid appearance. The mechanism of regression, unclarified at present, should be further studied.

Nodular fasciitis in the head and neck: CT and MR imaging findings.

BACKGROUND AND PURPOSE: The purpose of this study was to describe the CT and MR imaging findings of nodular fasciitis occurring in the head and neck region. METHODS: CT (n = 6) and MR (n = 4) images obtained from 7 patients (3 men and 4 women; mean age, 19.4 years; age range, 1-48 years) with surgically confirmed nodular fasciitis in the head and neck were retrospectively reviewed. All patients presented with a palpable mass in the head and neck that was noticed 1-3 months earlier: 5 in the face, one in the occipital scalp, and the remaining one in the supraclavicular fossa. We investigated the CT and MR imaging characteristics with emphasis on the location, size, internal content, margin, enhancement pattern, and signal intensity of the lesion. RESULTS: All lesions appeared as a discrete mass on imaging, ranging from 1.0 cm to 4.6 cm in diameter (mean, 2.2 cm). Six lesions, all of which appeared benign, were located in the subcutaneous tissue superficial to the deep cervical fascia. The remaining lesion was located deep to the temporalis muscle and showed an aggressive imaging appearance, markedly eroding the bony orbit and skull. Five lesions were solid, and 2 lesions were partly or completely cystic in appearance. Five lesions were well defined, whereas 2 lesions were ill defined. Four of 5 solid lesions showed moderate to marked diffuse enhancement, whereas the remaining lesion demonstrated mild enhancement. Two cystic lesions showed peripheral, nodular, or rim-like enhancement. Compared with muscle, both solid lesions had isointense signal intensity on T1-weighted images and hyperintense signal intensity on T2-weighted images, whereas the signal intensity of the solid portions of the deep-seated, partly cystic lesion was isointense on both T1-weighted and T2-weighted images. CONCLUSION: Although rare, nodular fasciitis occurs as a discrete solid or cystic mass in the head and neck, depending on the predominant stromal components. When one sees a head and neck mass with a superficial location and moderate to marked enhancement on CT and MR imaging, nodular fasciitis should be included in the differential diagnosis, especially in patients with a recently developed, rapidly growing mass and a history of recent trauma.

Clonal rearrangement of 15p11.2, 16p11.2, and 16p13.3 in a case of nodular fasciitis: additional evidence favoring nodular fasciitis as a benign neoplasm and not a reactive tumefaction.

This article describes a case of nodular fasciitis with the karyotype 47,XY,+4/46,XY,add(15)(p11.2), t(16;16)(p13.3;p11.2). The presence of clonal chromosomal abnormalities in this case, as well as in three previously reported cases, indicates that nodular fasciitis is a benign neoplasm and not a reactive lesion.

Necrotizing lesions of soft tissues: a review.

Necrotizing lesions of the soft tissues are grave entities not infrequently seen in daily surgical practice. They may occur with epidemic proportions after natural disasters, representing a serious challenge to the surgeon since they are characteristically associated with high mortality rates unless an early diagnosis is made and prompt aggressive surgical management is initiated. Necrotizing fasciitis is the currently accepted generic term to encompass into a single category the diverse syndromes of progressive gangrenous infections of the skin and subcutaneous tissues. Necrotizing fasciitis must be viewed as a clinical entity rather than a specific type of infection: it is a clinical infection most commonly caused by a mixed aerobic/anaerobic synergistic polymicrobial combination. Zygomycetes may appear as major causal organisms (mucormycosis) and they should be actively searched for. Initial diagnosis of necrotizing fasciitis is established through the characteristic physical signs. Gram stain, and, in some doubtful cases, through frozen-section tissue biopsy. Aggressive and urgent radical debridement is the key to survival, combined with wide-spectrum antibiotic therapy.

Necrotizing fasciitis in the pediatric age group: report of a case.

Necrotizing fasciitis is a rapidly progressive invasive soft tissue infection that is rarely seen in the pediatric population. In the majority of cases described in the medical literature, there has been an identifiable initiating event such as instrumentation or other trauma to the skin. Because of the rapid progression of the infection, the key to a successful outcome is early recognition and rapid initiation of definitive surgical management. A case is presented in which necrotizing fasciitis was seen in a child with no precipitating skin trauma.

Eosinophilic fasciitis after allogeneic bone marrow transplantation.

We describe a patient with eosinophilic fasciitis (EF) developing 8 months after an allogeneic bone marrow transplantation for acute myeloblastic leukemia. The patient responded to low dose prednisone. A full thickness skin-muscle-fascia biopsy detected the characteristic fascial changes of EF and distinguished it from other forms of chronic graft-versus-host-disease (GVHD). This distinction may be important since EF after bone marrow transplantation may occur more often and it may respond to treatment with low doses of prednisone whereas chronic GVHD usually requires more extensive immunosuppressive treatment.

[Fasciitis and Shulman's syndrome. A nosologic discussion]. / Fasciites et syndrome de Shulman. Un débat nosologique.

Eosinophilic fasciitis or Shulman's syndrome is linked to scleroderma, systemic sclerodermia as various types of localized sclerodermia, by a nosological relationship analyzed by the authors. But, excluding the one concerning eosinophilic fasciitis and deep morphea, the authors seem to believe that the current differences are still justified, since the clinical picture and the levels of the histological lesions vary from one entity to the other. The nosological debate concerning eosinophilic fasciitis demonstrates also that a histologically recognized fasciitis has no complete specificity. By far, it goes beyond sclerodermia. Inflammation of the fascia is possible in the course of disseminated lupus erythematosus and other connective tissue inflammations. It is also described beyond connective tissue inflammations during rhizomelic pseudo-polyarthritis, and mostly in the course of three diseases: Texier's disease, drepanocytosis, and the spanish toxic syndrome, where it may be associated to a hypereosinophilia.

Proliferative fibroblastic lesions. From hyperplasia to neoplasia.

It is rare to find a pathologist whose ideas and beliefs are so sound and pervasive as to influence our thinking decades after his writing. Yet such was the case with Dr. Stout, to whom we owe our basic classification of fibroblastic tumors, our concept of fibromatosis, and our diagnostic approach to fibrosarcomas. We need to continue the progress Dr. Stout made in these areas and to search for biochemical and molecular differences in these tumors with the hope that this knowledge will lead to new avenues for therapy.