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.

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.

Uncommon cutaneous neoplasms of the head and neck.

This article concentrates on the less-common cutaneous malignancies such as merkel cell, atypical fibroxanthoma, malignant fibrous histiocytoma, dermatofibrosarcoma protuberans, microcystic adnexal carcinoma, and sebaceous carcinoma. The clinical and histopathologic descriptions of each, most current and emerging etiologies, diagnosis, staging, treatment, and prognosis are discussed.

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.

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.

Dermoscopy of dermatofibromas: a prospective morphological study of 412 cases.

OBJECTIVE: To describe the dermoscopic features, including vascular structures and patterns associated with dermatofibromas in a large series of cases. DESIGN: Digital dermoscopic images of the prospectively collected dermatofibromas were evaluated for the presence of multiple structures and patterns. SETTINGS: Dermatofibromas were collected in the Departments of Dermatology of the Hospital de Sant Pau i Santa Tecla, Tarragona, Spain, and Hospital de Sant Llatzer, Palma de Mallorca, Spain. PATIENTS: A total of 412 dermatofibromas (from 292 patients) with complete documentation were collected. MAIN OUTCOME MEASURES: Frequency and intraobserver and interobserver agreement of the dermoscopic structures and patterns in dermatofibromas. RESULTS: A total of 19 morphological dermoscopic structures were evaluated. Pigment network was observed in 71.8% (3% atypical pigment network), white scarlike patch in 57.0%, and a white network in 17.7%. Different vascular structures were observed in 49.5% (dotted vessels in 30.6%). Ten dermoscopic patterns were observed. The most common pattern seen in our series (34.7% of cases) was central white patch and peripheral pigment network, but in 65.3% of the cases, dermatofibromas presented different patterns including simulators of melanoma. CONCLUSION: The most common pattern associated with dermatofibroma is the classic dermoscopic pattern (pigment network and central white patch), but this tumor has a wide range of presentations.

Incidence patterns of soft tissue sarcomas, regardless of primary site, in the surveillance, epidemiology and end results program, 1978-2001: An analysis of 26,758 cases.

Soft tissue sarcomas (STS) are a heterogeneous group of uncommon tumors that show a broad range of differentiation that may reflect etiologic distinction. Routine tabulations of STS are not morphology-specific. Further, the lack of inclusion of sarcomas arising in all organs in most standard evaluations underestimates the true rates. We analyzed the 1978-2001 Surveillance, Epidemiology and End Results program incidence rates of STS regardless of primary site, except bones and joints, using the 2002 criteria of the WHO classification. There were 26,758 cases available for analysis. Leiomyosarcomas accounted for 23.9%, malignant fibrous histiocytomas 17.1%, liposarcomas 11.5%, dermatofibrosarcomas 10.5%, rhabdomyosarcomas 4.6% and angiosarcomas 4.1%. Almost half (47.9%) of the sarcomas arose in the soft tissues, 14.0% in the skin and 7.0% in the uterus. Overall, incidence rates were highest among black women (6.26/100,000 woman-years) and the lowest among white women (4.60/100,000). Age-adjusted rates increased at 1.2% and 0.8% per year among white males and females, respectively, both trends statistically significant, while rates among blacks declined slightly. About 40% of leiomyosarcomas among women were uterine in origin, with a black/white rate ratio of 1.7. This rate ratio increased to 2.0 when we accounted for the lower prevalence of intact uteri among black compared to white women. Total STS rates rose exponentially with age. Rates for both uterine leiomyosarcoma and dermatofibrosarcoma increased rapidly during the childbearing years, peaking at about age 40 and 50, respectively. Incidence patterns of STS varied markedly by histologic type, supporting the notion that these tumors may be etiologically distinct.

Sarcomas of the oral and maxillofacial region: a review of 32 cases in 25 years.

Thirty-two cases of sarcomas involving the oral and maxillofacial region over a period of 25 years were reviewed. The age range was from 5 months to 77 years with a mean age of 42. The male to female ratio was 3:1. The sarcomas were located in the maxilla including the maxillary sinus (n= 13), mandible (n= 13), buccal mucosa (n= 3), temporomandibular fossa (n= 2), and submandibular region (n= 1). Histologically sarcomas were classified as osteosarcoma (n= 9), malignant fibrous histiocytoma (n= 7), rhabdomyosarcoma (n= 5), fibrosarcoma (n= 3), plasmacytoma (n= 2), leiomyosarcoma (n= 2), angiosarcoma (n= 2), liposarcoma (n= 1), and ameloblastic fibrosarcoma (n= 1). Surgical resection was performed in 29 cases. Local recurrence was found in 10 patients and metastasis in 11 patients. Metastases included five regional lymph node metastases and eight distant metastases. The survival of patients with local recurrence or metastasis was poor. Surgery is the most reliable treatment for sarcomas of the oral and maxillofacial region. Adequate excision with safety surgical margin as the initial therapy is important for better survival. The value of radiation therapy and/or chemotherapy is uncertain. The 5-year survival rate of primary cases was 61%.

[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.

Sclerosing haemangioma of the lung in a 4-year-old child.

Sclerosing haemangioma of the lung (SHL) should be recognised as a distinct clinicopathological entity. It is a benign neoplasm, probably of epithelial origin. Clinically, the tumour is asymptomatic and shows a striking preponderance in middle-aged women. SHL is often detected incidentally, as a round, well-defined homogeneous mass on routine chest radiograms. The diagnosis is based on pathohistologic examination of the biopsy material, therapy is surgical, and prognosis is excellent. We report a case of a 4-year-old boy with SHL, which is extremely rare in childhood.

Histopathological survey of neoplasms in flat-coated retrievers, 1990 to 1998.

Over the period from March 1990 to December 1998, veterinary surgeons in general practice were invited to submit tissues suspected of being neoplastic which had been removed from flat-coated retrievers. When possible, pedigree details were obtained from the owners. In addition, data were collected from flat-coated retrievers known to have suffered from a neoplastic condition and for which a histopathological report was available. A total of 1023 submissions was obtained from 782 dogs. These included 165 non-neoplastic lesions (16 per cent), 447 benign samples (44 per cent) and 411 malignant samples (40 per cent). Soft tissue sarcomas accounted for 55 per cent of the malignant samples (26 per cent of all tumour samples and 22 per cent of all submissions) with 63 per cent of them being diagnosed as undifferentiated. Carcinomas accounted for 20 per cent of malignant samples (8 per cent of all submissions). Of the benign tumours, cutaneous histiocytoma was the most common diagnosis (48 per cent of benign tumours, 25 per cent of all tumours and 21 per cent of all submissions).