Malignant fibrous neoplasms of long bones: analysis of the surveillance, epidemiology, and end results database from 1973 to 2015.

BACKGROUND: Malignant fibrous neoplasms (MFN) of long bones are rare lesions. Moreover, the prognostic determinants of MFN of long bones have not been reported. This study aimed to present epidemiological data and analyse the prognostic factors for survival in patients with MFN. MATERIALS AND METHODS: The Surveillance, Epidemiology, and End Results (SEER) programme database was used to screen patients with malignant fibrous neoplasms (MFN) of long bones from 1973 to 2015, with attention to fibrosarcoma, fibromyxosarcoma, periosteal fibrosarcoma and malignant fibrous histiocytoma. The prognostic values of overall survival (OS) and cancer-specific survival (CSS) were assessed using the Cox proportional hazards regression model with univariate and multivariate analyses. The Kaplan-Meier method was used to obtain OS and CSS curves. RESULTS: A total of 237 cases were selected from the SEER database. Malignant fibrous histiocytoma was the most common form of lesion in long bones. Multivariate analysis revealed that independent predictors of OS included age, stage, tumour size and surgery. Age, stage, tumour size and surgery were also independent predictors of CSS. Additionally, the most significant prognostic factor was whether metastasis had occurred at the time of initial diagnosis. CONCLUSION: Among patients with MFN of long bones, age (> 60 years), tumour size (> 10 cm), distant stage, and non-surgical treatment are factors for poor survival.

Incidence rate and clinicopathological features of 62 atypical fibroxanthomas in a North-Western Spanish population.

BACKGROUND/OBJECTIVES: Atypical fibroxanthoma (AFX) is a mesenchymal neoplasm of unknown incidence. It has been determined that AFX is a tumour with low aggressiveness as long as it is properly diagnosed. Our objectives were to exclude pleomorphic dermal sarcomas or other skin tumours incorrectly diagnosed as AFX in our centre after applying strict diagnostic criteria and to assess the behaviour of appropriately diagnosed AFX. METHODS: We conducted an observational retrospective analysis of 73 patients diagnosed with AFX in our centre between 1998 and 2018. After selecting cases fulfilling AFX criteria, we made an analysis of predictive factors for local recurrence. Crude and sex-adjusted incidence rates were calculated. RESULTS: Out of 73 cases, 62 were eventually diagnosed as AFX. We examined for absence of tumour necrosis, lymphovascular or perineural invasion and infiltration of deep structures. Cytokeratin AE1-AE3, desmin and CD34 were negative in all cases. The remaining tumours were reclassified. The incidence of AFX in our health-care area was estimated at 0.59 cases every 100 000 inhabitants per year. In our series, 72.6% of the patients were men with mean age at diagnosis of 81 years. Average tumour diameter was 12 mm. The most common location was head and neck (96.8%). Only four local recurrences were detected over a mean of 47-month follow-up. CONCLUSIONS: We report a series of AFX in our health-care area. We verify its indolent course when it is properly diagnosed.

Atypical Fibroxanthoma: The Washington University Experience.

BACKGROUND: Atypical fibroxanthoma (AFX) is a rare dermal neoplasm typically occurring on sun-exposed skin in the elderly. As AFX remains a diagnosis of exclusion, updated characterization and treatment assessments are necessary to support informed diagnosis and management. OBJECTIVE: Characterization of contemporary AFX and surgical outcomes by Mohs micrographic surgery (MMS) and conventional local excision (LE). METHODS: Retrospective cohort analysis of all cases of AFX at our institution from January 2000 through July 2016. RESULTS: Among 75 cases with median age at diagnosis 73 years, most occurred on the head and neck (68) independent of age. Most treated cases (42) underwent MMS alone, with median tissue removal greater for LE (2.6 cm, 4.5 cm) than MMS (0.6 cm, 1.2 cm). Over a median 26 months of follow-up, 6 recurrences were observed among 50 cases, with metastases in 2 cases. Intent-to-treat recurrence rates were 3.4% for MMS and 25% for LE. One nonrecurrent and 2 recurrent cases received revised diagnoses after initial treatment, yielding a true recurrence rate of 8.5%. CONCLUSION: Despite diagnostic confounding by similar pathologies, surgical treatment of AFX remains effective. Tissue-sparing resection by MMS affords the potential for cosmetic and reconstructive advantage, without compromising recurrence compared with conventional excision.

Cellular Dermatofibroma: Clinicopathologic Review of 218 Cases of Cellular Dermatofibroma to Determine the Clinical Recurrence Rate.

BACKGROUND: Cellular dermatofibromas, a variant of dermatofibroma, are reported to recur at rates of 26% to 50%. OBJECTIVE: To determine whether there are distinct clinical or histological differences between cellular dermatofibromas that recur versus those that do not. To determine recurrence rates in a real-world clinical setting. MATERIALS AND METHODS: A retrospective analysis of the medical records and skin biopsies of cellular dermatofibroma in the University of Utah Health system between December 2011 and 2016. Clinical and dermatopathological features were evaluated to find distinct differences between the cellular dermatofibromas that recurred compared with those that did not. RESULTS: There were no significant differences in histology between the primary lesions in recurrent and nonrecurrent cases. One factor that seemed to be associated with a greater likelihood of recurrence was an initial lesion size greater than 1 cm. The authors' data suggest that if the margins are involved on initial biopsy, there is a 10% chance of recurrence. This percentage is far less than the 26% to 50% reported in the past literature. CONCLUSION: If a patient presents with a cellular dermatofibroma larger than 1 cm and positive margins at initial biopsy, a careful discussion should be had between the provider and patient about the low risk of local recurrence.

The significance of crystalline/chrysalis structures in the diagnosis of melanocytic and nonmelanocytic lesions.

BACKGROUND: Crystalline/chrysalis structures (CS) are white shiny streaks that can only be seen with polarized dermatoscopy. OBJECTIVES: We sought to estimate the prevalence and assess the clinical significance of CS in melanocytic and nonmelanocytic lesions. METHODS: This was a prospective observational study in which dermatoscopic assessment of lesions was recorded in consecutive patients examined during a 6-month period. In addition, a data set of biopsy-proven melanomas was retrospectively analyzed. RESULTS: In all, 11,225 lesions in 881 patients were prospectively examined. Retrospectively, 229 melanomas imaged with polarized dermatoscopy were analyzed. In the prospective data set, a median of 12.7 lesions (range, 1-54) were evaluated per patient. None of clinically diagnosed Clark nevi (n = 9750, 86.8%) demonstrated CS. Overall, CS were observed in 206 (1.8%) lesions, most commonly dermatofibromas and scars among nonbiopsied lesions. A total of 265 (2.4%) lesions were biopsied, including 20 melanomas and 36 nevi. Among biopsied malignant lesions, CS were most commonly observed in basal cell carcinoma (47.6%) and invasive melanomas (84.6%). Melanomas were more likely to have CS than biopsied nevi (odds ratio = 9.7, 95% confidence interval 2.7-34.1). In the retrospective data set, CS were more commonly observed among invasive melanomas (41%) compared with in situ melanomas (17%) (odds ratio = 3.4, 95% confidence interval 1.9-6.3, P < .001). The prevalence of CS correlated with increased melanoma thickness (P = .001). LIMITATIONS: Biopsied lesions represent a small percentage of the total number of lesions evaluated. CONCLUSION: Among biopsied malignant lesions, CS are most commonly observed in basal cell carcinoma and invasive melanomas and rarely seen in nevi. In melanoma, CS may reflect increased tumor thickness and progression.

Genetic skin diseases predisposing to basal cell carcinoma.

Basal cell carcinoma (BCC) is the commonest cancer in humans. Predisposing factors reflect common genetic variations and environmental influences in most cases. However, an underlying Mendelian disorder should be suspected in a specific subset of patients, namely those with multiple, early onset lesions. Some specific conditions, including Gorlin, Bazex-Dupré-Christol and Rombo syndromes, and Xeroderma Pigmentosum, show BCC as a prominent feature. In addition, BCC may represent a relatively common, although less specific, finding in many other genodermatoses. These include disorders of DNA replication/repair functions (Bloom, Werner, Rothmund-Thomson and Muir-Torre syndromes), genodermatoses affecting the folliculo-sebaceus unit (Brooke-Spiegler, Schöpf-Schulz-Passarge and Cowden syndromes), immune response (cartilage-hair hypoplasia and epidermodysplasia verruciformis) and melanin biosynthesis (oculocutaneous albinism and Hermansky-Pudlak syndrome), and some epidermal nevus syndromes. Further conditions occasionally associated with BCCs exist, but the significance of the association remains to be proven.

Clinical and histological patterns of dermatofibroma without gross skin surface change: A comparative study with conventional dermatofibroma.

BACKGROUND: Dermatofibroma sometimes clinically presents as a nodular lesion without gross skin surface change. Clinicopathologic features of this variant of dermatofibroma have not been evaluated. AIMS: To assess clinicopathologic features of dermatofibroma presenting as a subcutaneous nodule. METHODS: This study reviewed the clinical and histological features of 42 cases of subcutaneous dermatofibromas and compared them with 95 cases of conventional dermatofibroma. RESULTS: Dermatofibroma without gross skin surface change was associated with a shorter pre-diagnosis duration than conventional dermatofibroma. Increase in size during the pre-diagnosis period was significantly more frequent in the conventional type. In addition, these dermatofibromas were more likely than the conventional type to occur in the head and neck region. Although tumor depth was deeper than in the conventional type, less than half of the dermatofibromas without gross skin surface change were found histologically to be "subcutaneous" or "deep-penetrating dermatofibroma". Subcutaneous extension was more frequent in these dermatofibromas while focal stromal hyalinization and hemosiderin deposits were more common in the conventional type. LIMITATIONS: This study is a retrospective, single center design. CONCLUSION: The present study suggests that dermatofibroma without gross skin surface change is a variant type with distinct clinical and histological features that distinguish them from conventional dermatofibroma.

Atypical fibroxanthoma: a series of 56 tumors and an unexplained uneven distribution of cases in southeast Germany.

BACKGROUND: Atypical fibroxanthoma is a rare mesenchymal tumor of the head and neck region. METHODS: We analyzed the files of 3 large dermatology hospitals from the years 2001 to 2013 in southeast Germany. RESULTS: We identified 53 patients (56 tumors) with a male predominance. The mean age was 78.0 years ± 8.3 years. Mohs surgery was performed in all cases resulting in complete remission in 45 patients. Five patients had a relapse within 2 years, and 4 developed metastases. None of the tumors with a safety margin of ≥2 cm relapsed. The majority of cases were found in the Dresden region. CONCLUSION: Atypical fibroxanthoma demonstrates an uneven geographic distribution in southeast Germany that demands further investigations. Mohs surgery with ≥2 cm safety margins is the treatment of choice. A regular follow-up is recommended.

Long-term risk of sarcoma following radiation treatment for breast cancer.

Between 1954 and 1983, 7620 patients were treated for breast carcinoma at Institut Gustave Roussy (France). Of these patients, 6919 were followed for at least 1 year. Out of these, 11 presented with sarcomas thought to be induced by irradiation, 2 of which were Steward-Treves Syndrome, and 9 of which were sarcomas within the irradiated fields. All histological slides were reviewed and a comparison with those of breast cancer was done. The sites of these sarcomas were: parietal wall, 1 case; second costal cartilage, 1 case; infraclavicular region, 1 case; supraclavicular region, 2 cases; internal third of the clavicle, 2 cases; axillary region 2 cases; and the internal side of the upper arm (Stewart-Treves syndrome), 2 cases. The median age of these 11 patients at the diagnosis of sarcomas was 65.8 (49-83). The mean latent period was 9.5 years (4-24). Three patients underwent radical mastectomy and nine modified radical mastectomy. Only one patient received chemotherapy. The radiation doses received at the site of the sarcoma were 45 Gy/18 fr. for 10 cases and 90-100 Gy for 1 case (due to overlapping between two fields). The histology was as follows: malignant fibrous histiocytoma, 5 cases; fibrosarcoma, 3 cases; lymphangiosarcoma, 2 cases; and osteochondrosarcoma, 1 case. The median survival following diagnosis of sarcoma was 2.4 years (4 months-9 years). Two patients are still alive: one with recurrence of her breast cancer, the other in complete remission, with 7 and 3 years follow-up, respectively. All other patients died from their sarcomas. The cumulative incidence of sarcoma following irradiation of breast cancer was 0.2% (0.09-0.47) at 10 years. The standardized incidence ratio (SIR) of sarcoma (observed n# of cases (Obs)/expected n# of cases (Exp) computed from the Danish Cancer Registry for the same period) was 1.81 (CI 0.91-3.23). This is significantly higher than one, with a p = 0.03 (One Tailed Exact Test). The mean annual excess (Obs-Exp)/100.000 person-years at risk during the same period/(100,000) was 9.92. This study suggests that patients treated by radiation for breast cancer have a risk of subsequent sarcomas that is higher than the general population. However, the benefit from adjuvant radiation therapy in the treatment of breast cancer exceeds the risk of second cancer; therefore, the potential of radiation-induced sarcomas should not be a factor in the selection of treatment for patients with breast cancer.

Angiomatoid malignant fibrous histiocytoma. A follow-up study of 108 cases with evaluation of possible histologic predictors of outcome.

Described by Enzinger in 1979, angiomatoid malignant fibrous histiocytoma is a distinctive tumor of adolescence and early adult life characterized by sheets of relatively bland rounded or spindled cells separated by areas of cystic hemorrhage and surrounded by a prominent inflammatory infiltrate and often a fibrous pseudocapsule. On the basis of the original 41 cases, the tumor has been considered a low-grade malignancy. We are reporting the clinicopathologic findings and follow-up information of 108 new cases of angiomatoid malignant fibrous histiocytoma to determine the long-term behavior of this tumor and whether various histologic features (atypia, mitoses, infiltrative borders, and inflammatory infiltrate) are useful in predicting outcome. Follow-up information was obtained in 94 (87%) cases. Local recurrences developed in 11 patients (12%), all of whom were cured by re-excision. The initial excision in all patients developing local recurrence appeared to be incomplete. Local recurrence was statistically associated with irregular tumor border and head and neck location. Five patients developed metastases. Four had only local metastases, which responded to surgery, whereas the fifth patient developed presumed pulmonary and cerebral metastases and died. The development of both local and distant metastases was correlated with invasion into the deep fascia or muscle but not to various histologic parameters such as mitotic rate and pleomorphism. We conclude that angiomatoid malignant fibrous histiocytoma is intrinsically a low-grade tumor, and assessment of various histologic parameters or grading provides little or no additional prognostic information. Because death from disease occurred in only one patient (1%) in our series, it seems reasonable to reclassify angiomatoid malignant fibrous histiocytoma with fibrohistiocytic tumors of intermediate malignancy rather than with the conventional malignant fibrous histocytoma, the majority of which are high-grade sarcomas.

Follicular basal cell hyperplasia overlying dermatofibroma.

Follicular basal cell hyperplasia (FBCH) overlying dermatofibroma represents aborted or impeded pilar differentiation. Historically, this hyperplasia has been misinterpreted as basal cell carcinoma. In a large series of dermatofibroma (258 cases), those that contained primitive or malformed follicular structures over the lesion (59 cases) were compared with those without such elements (199 cases). Statistical analysis of various clinicopathologic features showed that FBCH was significantly associated with younger age, trunk location, hypercellular dermatofibroma, loss of a Grenz zone, clear cell hyperplasia, and seborrheic keratosis-like change. There was an inverse correlation between epidermal atrophy, lichen simplex chronicus-like change, and lower extremity location with FBCH. Histologic features favoring a diagnosis of FBCH over basal cell carcinoma are the focal nature and superficial location of the lesion, lack of cytologic atypia and mitoses, recognizable components of hair follicle differentiation, focal condensation of mesenchymal cells around basal cell proliferation, and the association of epidermal hyperplasia. Our findings suggest that FBCH, clear cell hyperplasia, and seborrheic keratosis-like change all represent an expression of follicular differentiation overlying dermatofibroma.

Cytometry and morphometry of malignant fibrous histiocytoma of the extremities. Prediction of metastasis and mortality.

Flow cytometry and nuclear morphometry were compared with traditional pathologic grading techniques for predicting the course of malignant fibrous histiocytoma of the extremities. Clinical, pathologic, and flow/morphometric variables from 53 cases were tested by Cox regression for prediction of distant recurrence and mortality. Tumor grading based on extent of tumor necrosis was a significant predictor for both disease-free survival (p = .014) and overall survival (p = .003). The fraction of nuclei in the S + G2M segment of DNA histograms was significant for disease-free survival (p = .007), and remained significant (p = .033) in a joint Cox model with necrosis-based grade (p = .004 for the bivariate model). Relative risk for recurrence varied nearly 10x between the 10th and 90th percentiles of grade and (S + G2M)1/2. Overall survival was predicted by a nuclear shape feature termed "R" (p = .000008), the casewise difference (residual) between expected and observed nuclear perimeter as a function of average Feret diameter. In a bivariate Cox model, relative risk of mortality varied 35x between the 10th and 90th percentiles of grade and R. Cytometric and morphometric data contain information about recurrence-free and overall survival beyond that available from more usual clinical and pathologic features. It seems likely that nuclear morphometry, in particular, will prove to be a useful aid for estimating the prognosis of patients with malignant fibrous histiocytoma of the extremities.

Prosthetic survival and clinical results with use of large-segment replacements in the treatment of high-grade bone sarcomas.

We evaluated the long-term clinical results and the survival of the prostheses in eighty-two patients who had had a limb-sparing procedure by means of the implantation of a large-segment prosthesis. All patients had had a high-grade bone sarcoma of the distal, middle, or proximal part of the femur; the proximal part of the humerus; the proximal part of the tibia; or the pelvis. The duration of follow-up ranged from two to twelve years (median, three and one-half years). Function was evaluated with the revised 30-point classification system of the Musculoskeletal Tumor Society. The survival of the prostheses was analyzed with regard to several variables with use of Kaplan-Meier survival estimates. Sixty-eight patients were alive at the latest follow-up evaluation. The survival rate of the prostheses was 83 per cent at five years and 67 per cent at ten years. Twelve prostheses were revised, and eleven revisions were successful. The rate of revision was highest (six of thirteen) in the patients who had had a tumor of the proximal part of the tibia. In contrast, only three (10 per cent) of the thirty-one patients who had had a tumor of the distal part of the femur and three (10 per cent) of the twenty-nine who had had a tumor of the proximal part of the humerus had a revision. Eleven patients (13 per cent) had an infection, which necessitated an amputation in six. Five patients (6 per cent) had a local recurrence, and nine patients (11 per cent), including the six already mentioned, ultimately needed an amputation. Patients who had had a tumor of the proximal part of the humerus had the highest functional scores, while those who had had a tumor of the proximal part of the tibia had the lowest scores. Large-segment prostheses were a good reconstructive option for the treatment of high-grade bone sarcomas in our patients. The rates of long-term survival of the prostheses were acceptable and the functional results were good or excellent after this form of treatment at most of the anatomical sites at which they were used.

Fibrosarcoma of the head and neck.

A review of 118 archival cases originally diagnosed as fibrosarcoma of the head and neck is presented. Sites of involvement were the sinonasal region, upper aerodigestive tract, skin, and soft tissues. Review of the pathologic specimens applying strict histopathologic criteria including immunohistochemical techniques reclassified many of the tumors as malignant fibrous histiocytoma, monophasic synovial sarcoma-like neoplasm, or dermatofibrosarcoma protuberans. Improved clinical outcome correlated significantly with lesser tumor size, lower histologic grade, and negative surgical margins. Location in the skin or soft tissue tended to convey improved survival, although not to a level of statistical significance.

[Malignant fibrous histiocytoma of bone (MFH)--an new tumour? ]. / Das maligne fibröse Histiozytom des Knochens (MFH)--eine neue Tumorentität?

Malignant fibrous histiocytoma of bone (MFH) is a histologically defined bone tumour, which can be distinguished from osteo- and fibrosarcomas. The present paper deals with the question whether MFH can be distinguished from these tumours on clinical grounds. One hundred and fifty-seven previously published, and four additional cases have been analysed with respect to their localisation and their biological, clinical and radiological behaviour and their prognosis. It was found that MFH can be distinguished from osteosarcomas on clinical grounds and that this is significant in relation to treatment; its distinction from fibro-sarcomas is clinically not justified.

Rhinopharyngeal angiofibroma in the pediatric age group. Clinical-statistical contribution.

The authors had the chance of treating two cases of rhinopharyngeal angiofibroma before the age of puberty, and were thus stimulated to re-examine the incidence of such neoplasms in the pediatric age group, i.e. under 12 years of age. From their clinical statistical studies, and in the light of their personal experience, they were able to make the following observations. 12.9% of angiofibromas occur by the age of 12 years. These tumours present a shorter period of symptomatological evidence with respect to those which occur later. They are histologically characterized by a greater richness of cellular and vascular components with respect to those of a fibrous nature. Among the equipment used in radiology, computerized axial tomography showed itself extremely useful. The paralateronasal path is the most commonly used among surgical paths, as it is able to throw more light on tumour. The frequency with which the neoplastic mass relapses during operation is proportionally greater the earlier the primitive tumour occurs.

[Frequency of genetic diseases and cancer antecedents in 493 adults with visceral or soft tissue sarcomas]. / Fréquence des génopathies et des antécédents carcinologiques chez 493 adultes atteints de sarcomes viscéraux ou des tissus mous.

Little is known about epidemiology of adults soft tissue and visceral sarcomas (ASTS). The frequency of previous cancers and associated genetic diseases has been analyzed out of 493 ASTS, treated between 1997 and 2002 at Oscar Lambret Cancer Center. Median age is 51, sex ratio is close to 1. Liposarcomas and malignant fibrous histiocytofibromas are the two main types (respectively 104 and 86 cases). Upper and lower limbs are the two main locations (respectively 176 and 75 cases). Fifteen patients had associated genetic disease, including 12 cases of Recklinghausen diseases. 7 out of those 15 patients have neurosarcoma. 30 patients have previous cancers, including 7 breast cancers, 3 lymphomas and 3 chronic lymphocytic leukemias. Four out of those 30 patients have two different previous cancers. 13 patients have radiation-induced sarcomas, after an average 10-year-period, and an average dose of 53 Gy. Undifferenciated sarcomas are the main histologic type (8/13), followed by angiosarcomas (2/13). Radiation-induced sarcomas are located in the chest wall (7/13), in pelvis (2/13) and head and neck (2/13). Those sarcomas are high grade (10 grade III tumours). ASTS epidemiology is complex with different risk factors depending on histologic type.