Re-irradiation using proton therapy for radiation-induced secondary cancer with Li-Fraumeni syndrome: A case report and review of literature.

Li-Fraumeni syndrome (LFS) is a genetic disease that is hypersensitive to radiotherapy. Proton therapy (PT) was strongly recommended for pediatric and radiation-sensitive tumors. However, there is little information on PT for LFS. The patient was a 7-year-old girl with LFS who was diagnosed with radiation-induced right shoulder blade osteosarcoma and left chest wall malignant fibrous histiocytoma. Both tumors were in the area that had previously been irradiated (36-45 Gy by photon radiotherapy). Sixty-six GyE in 30 fractions was planned for both tumors. We set the clinical target to the minimum gross tumor volume. To comprehensively assess any adverse events, PT was conducted under hospital administration. Cisplatin was used as simultaneous combination chemotherapy. Although administration of granulocyte-colony stimulating factor was necessary for myelosuppression by chemotherapy, PT was completed without interruption. Acute radiation toxicity was observed as Grade 1 dermatitis. The dermatitis became exacerbated 2 weeks after PT but subsequently improved with conservation treatment alone. Twenty-three months after PT, magnetic resonance imaging showed an increase in the tumor on the right shoulder. A histological examination was not conducted as the family declined, but secondary cancer was suggested rather than recurrent osteosarcoma, as the tumor developed mainly from the soft tissue. Additional surgical treatment and radiotherapy were not indicated, and the patient died of tumor progression and sepsis caused by myelosuppression 27 months after undergoing PT. Up to 23 months after PT, there were no signs of Grade 2 or more late toxicities. This represents the first reported case of PT for a patient with LF to treat radiation-induced secondary cancer.

Estudio prospectivo de la combinación de láser de alejandrita Q-Switched de 755nm y el láser V Beam de colorante pulsado de 595nm en dermatofibromas / Prospective Study of Q Switched Alexandrite Laser 755 nm and V Beam Pulsed Dye Laser 595 nm in Dermatofibroma

El uso de los láseres de colorante pulsado (pulse dye laser [PDL]) y de dióxido de carbono (CO2) para el tratamiento de los dermatofibromas (DF) ha demostrado tener buenos resultados clínicos. Un total de 23 DF fueron tratados en dos sesiones con el láser de alejandrita Q-Switched de 755 nm (7,5 J/cm2, 3 mm, 50 ms), dejando entre cada sesión un intervalo de 4semanas. Para el eritema residual se utilizó el láser V Beam PDL con una longitud de onda de 595nm (10-11 J/cm2, 7 mm, 1,5 ms). En 9 de las pacientes se observó una atenuación parcial de la coloración marrón y en 14 de ellas una desaparición completa de la misma. Las pacientes refirieron un grado elevado de satisfacción después de haber realizado el tratamiento. Así mismo, 15 de las pacientes notaron una disminución en el endurecimiento de los DF. En el examen dermatoscópico previo al tratamiento, en todos los DF se observó la presencia de una red de pigmento. Esta red de pigmento desapareció en todos los casos tras la aplicación del láser. La combinación del láser V Beam PDL y del láser de alejandrita Q-Switched en los DF constituye una alternativa terapéutica adecuada que, además de buenos resultados cosméticos, ha conseguido un elevado grado de satisfacción por parte de los pacientes

Pulsed dye and carbon dioxide lasers have been applied in dermatofibroma with clinical improvement. We treated 23 dermatofibromas two times at a 4-week interval with Q-Switched alexandrite laser 755 nm (7.5 J/cm2, 3 mm, 50 ms). V Beam pulsed dye laser with a wavelength of 595nm was used for the residual erythema (10-11 J/cm2, 7 mm, 1.5 ms). A partial attenuation of brown colour was observed in 9 patients and complete disappearance of brown colour in 14 patients. Patient satisfaction was very high. Fifteen patients felt a decrease in hardening of dermatofibroma after treatment. A pigment network in dermoscopy was observed in all patients before treatment and no one after treatment. A combined treatment using both V Beam pulsed dye laser and Q-Switched alexandrite laser may be a therapeutic option to reduce the aesthetic effect of dermatofibroma with a high patient satisfaction and good cosmetic outcomes

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.

Conjunctival Benign Fibrous Histiocytoma in a 57-year-old man.

Purpose. To report a case of benign fibrous histiocytoma of the conjunctiva involving the cornea, an uncommon ocular surface tumor. Methods. A 57-year-old patient came in our service complaining of a progressively enlarging conjunctival mass temporally to the limbus and invading the adjacent cornea of the left eye. Results. The approach consisted in surgical excision followed by cryotherapy on the edges and on the base of the excision site and amniotic membrane patch reconstruction of the ocular surface defect. Pathologic examination and immunohistochemistry were performed in order to establish the diagnosis. No recurrences appeared in 8 months of follow up. Conclusions. Fibrous histiocytoma might be easily misdiagnosed as it is exceedingly rare. Complete resection with careful inspection of edges is advised. Cryotherapy at the base and borders of the resection site is recommended as both benign and malignant tumors can show recurrence. Amniotic membrane should always be regarded as an efficient option in reconstruction of broad surface defects after tumor resection. Abbreviations: FH = fibrous histiocytoma, CIN = corneal intraepithelial neoplasia, SSCA = squamous cell carcinoma, AM = amniotic membrane, MMC = topical mitomycin-C, 5-FU = 5-fluorouracil, BCVA = best corrected visual acuity.

Pulmonary sclerosing pneumocytoma (sclerosing haemangioma): Radical radiation therapy.

We present a case of pulmonary sclerosing pneumocytoma (PSP) - (Formerly known as pulmonary sclerosing haemangioma) which was successfully treated with definitive radical external beam radiation therapy (EBRT). To our knowledge, such a treatment response has not been described in the literature.

Sclerosing angiomatoid nodular transformation of the spleen during pregnancy: Diagnostic challenges and clinical management.

We report the first case of sclerosing angiomatoid nodular transformation (SANT) of the spleen diagnosed during pregnancy, discussing differential diagnosis, immunohistochemical profile and treatment. A G2P1 37-year-old woman presented during the 19th week of gestation because of pruritus at lower limbs. To exclude cholestasis, an abdominal ultrasound and whole body magnetic resonance were performed and a single solid lesion with intrinsic vascularization was identified. Therefore, at 22 weeks gestation, after normal fetal assessment, the patient was referred for a splenectomy. No further treatment was suggested and the patient gave birth at 42 weeks gestation with a spontaneous delivery. Distinguishing SANT from other vascular neoplasms of the spleen during pregnancy is a difficult task. Surgical excision should be performed to exclude malignancy and to resolve symptoms, if present.

¿Histiocitosis pulmonar? o ¿tuberculosis pulmonar? en un paciente con infiltración histiocitaria cutánea / No disponible

Se presenta un caso de una paciente de 62 años, remitida desde Dermatología a nuestra consulta por presentar una lesión pápulo-nodular infiltrada eritematosa en mejilla izquierda con resultado en la biopsia de una infiltración dérmica y de tejido celular subcutáneo por histiocitos y ocasionales células gigantes multinucleadas con material microvacuolar. En la TC torácica para estudio de extensión aparecen infiltrados parenquimatosos sospechoso de histiocitosis pulmonar. Informándose en el estudio microbiológico del BAS como tinción de Ziehl-Nielsen positiva

A case of a female patient aged 62, sent from Dermatology to our office due infiltrated erythematous papule-nodular lesion in the left cheek with biopsy results in a dermal infiltration and subcutaneous tissue histiocytes and occasional giant cells were present with microvacuolar material. In the thoracic CT for suspected parenchymal extension study of pulmonary histiocytosis appear infiltrates. Micribiologico informing the study of BAS as Ziehl-Nielsen positive

¿Por qué la cirugía micrográfica de Mohs está infrautilizada en el tratamiento del dermatofibrosarcoma protuberans infantil? / Why Is Mohs Micrographic Surgery Underused in the Treatment of Dermatofibrosarcoma Protuberans in Children?

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Atypical Fibroxanthoma - Histological Diagnosis, Immunohistochemical Markers and Concepts of Therapy.

BACKGROUND: Atypical fibroxanthoma (AFX) is an uncommon, rapidly growing cutaneous neoplasm of uncertain histogenesis. Thus far, there are no guidelines for diagnosis and therapy of this tumor. PATIENTS AND METHODS: We included 18 patients with 21 AFX, and 2,912 patients with a total of 2,939 AFX cited in the literature between 1962 and 2014. RESULTS: In our cohort, excision with safety margin was performed in 100% of primary tumors. Local recurrences were observed in 25% of primary tumors and parotid metastases in 5%. Ten-year disease-specific survival was 100%. The literature research yielded 280 relevant publications. Over 90% of the reported cases were negative for cytokeratins, S100, desmin and human melanoma black 45 (HMB-45). Recurrent AFX was reported in 7.6% and metastasizing AFX in 2.75% cases. No significant differences in the recurrence and survival rates following wide local excision versus Mohs microsurgery were observed. Twenty-year disease-specific survival rate was 97.8%. CONCLUSION: A well-selected panel of immunohistochemical markers is necessary to establish AFX diagnosis with sufficient certainty. Adequately treated, AFX has an excellent prognosis, but long-term follow-up is recommended due to the potential for aggressive behavior.

Malignant transformation in monostotic fibrous dysplasia: clinical features, imaging features, outcomes in 10 patients, and review.

Malignant transformation in fibrous dysplasia (FD) is uncommon. The purpose of this study was to investigate clinical and imaging features, and outcomes of malignant transformation in monostotic FD.Data for 10 pathologically confirmed malignant transformations in monostotic FD from January 2005 to December 2013 were retrospectively reviewed. Patient data were recorded, and radiographs (n = 10), computed tomography (CT) (n = 5), magnetic resonance (MR) (n = 4), and bone scintigrams (n = 10) were evaluated for lesion location, margin, cortical destruction, marrow involvement, periosteal reaction, and soft tissue mass by 2 musculoskeletal radiologists with agreement by consensus. Clinical features, management, and prognosis were also analyzed for each of the 10 cases.There were 8 male and 2 female patients (mean age 46.5 ±â€Š15.9 years). The affected sites were the femur (n = 4), humerus (n = 2), tibia (n = 3), and ilium (n = 1). Five cases had received previous surgery and 5 cases had no history of surgery. No patients had been given prior irradiation treatment. For the 5 cases with surgery, radiographs and CT showed purely osteolytic lesions with poor margination in the curettage area (n = 5), cortical destruction (n = 5), obvious soft tissue mass (n = 1), and mineralization (n = 2). For the 5 cases without surgery, radiographs and CT identified poorly marginated, osteolytic lesions within or near the area with "ground-glass" opacity (n = 4), cortical erosion (n = 4), and mineralization (n = 2). Magnetic resonance imaging (MRI) also identified lesions with heterogeneous signal intensity and pronounced enhancement. Bone scintigraphy revealed eccentric increased uptake of radionuclide in monostotic lesion (n = 10). Pathology reports revealed osteosarcoma (n = 7), fibrosarcoma (n = 2), and malignant fibrous histiocytoma (MFH) (n = 1). At the end of the study, 1 patient died from tumors, 1 patient was alive with lung metastasis, 1 patient experienced recurrence, and 7 patients were alive without recurrence.Patients with FD and a history of surgery should be followed up, for the osteolytic lesions in the operative areas strongly indicate the malignant transformation. The radiographic feature of FD-related malignancies is poorly marginated, mineralized, and osteolytic lesions with cortical destruction. Further investigations are needed to explore the pathogenesis of malignancies in FD and to establish optimal therapeutic strategies.

Treatment of oral hemangioma with sclerotherapy: case report / Tratamento de hemangioma oral com escleroterapia: relato de caso

Hemangiomas are benign neoplasms that are common in the head and neck, but relatively rare in the oral cavity. They can cause esthetic and functional impairment, depending on location. The most common site is the upper lip, but they can occur in other areas, such as the tongue, buccal mucosa and palate. Treatment is primarily dependent on correct diagnosis of the lesion and on its anatomic location. The purpose of this article is to provide a description of a case of a hemangioma on the upper lip, treated by therapeutic sclerosis with monoethanolamine oleate (Ethamolin®), covering clinical characteristics and methods for diagnosing these lesions. Precise diagnosis and appropriate therapeutic management resulted in satisfactory esthetic and functional results, with total regression of the lesion and no signs of relapse at 1-year follow-up...

O hemangioma é uma neoplasia benigna comum na região de cabeça e pescoço, e é relativamente rara na cavidade oral, podendo causar prejuízo estético e funcional a depender da sua localização. Sua localização mais frequente é o lábio superior, mas pode ocorrer em outras regiões, como língua, mucosa jugal e palato. O seu tratamento depende, principalmente, do correto diagnostico da lesão, bem como da localização anatômica da mesma. A proposta deste artigo é relatar um caso de hemangioma em lábio superior tratado com esclerose terapêutica com oleato de monoetanolamina (Ethamolin®), considerando as características clínicas e os métodos de diagnóstico desta lesão. Por meio de um diagnóstico preciso e uma conduta terapêutica adequada, o caso apresenta-se com um acompanhamento de um ano, sem sinais de recidiva, e com um resultado estético funcional satisfatório, com regressão total da lesão...

Rare type of bladder cancer: malign fibrous histiocytoma.

Malignant fibrous histocytoma (MFH) is the most common soft tissue sarcoma in adults. Urinary tract is a very rare location for MFH. Involvement of the bladder is more common in males and at the 6th decade of life. A case of MFH of the bladder with poor prognosis is presented. Prognostic factors for MFH are tumor grade, amount of invasion, age, tumor size, and histological type. Survival rate is very low and 3-year disease specific survival is approximately 40%.

Treatment of intracapsular femoral neck lesions: aggressive or conservative surgery?

Benign fibrous histiocytomas (BFHs) and non-ossifying fibromas (NOFs) are benign bone tumors that are clinically different, but histologically similar. Most benign lesions are treated with curettage and grafting; however, some misdiagnosed cases are treated with more aggressive procedures, including arthroplasty. Herein, we present a 21-year-old patient that presented with a 1-year history of right hip pain. Anteroposterior X-ray showed a centrally located lesion in the femoral neck that was intracapsular and localized subcortically. To the best of our knowledge, the literature does not include any reposts of BFH or NOF localized at the intracapsular hip. In addition, localization of and treatment options for intracapsular hip lesions are clarified.

Treatment of a symptomatic dermatofibroma with fractionated carbon dioxide laser and topical corticosteroids.

Dermatofibromas are benign skin lesions that may be treated if symptomatic or for cosmetic concerns. We present a case of an African American woman with an enlarging, pruritic dermatofibroma on the thigh that was treated with fractionated carbon dioxide (CO2) laser three times approximately 5 weeks apart. Between laser treatments, topical corticosteroids were applied to the lesion for a total of 13 weeks. The dermatofibroma completely flattened and became asymptomatic within 1 month after the final laser treatment. We hypothesize that the fractionated CO2 laser ablated a portion of the stromal component of the lesion and introduced microscopic channels that facilitated deeper penetration of the topical corticosteroids into the lesion. This is the first reported case demonstrating the successful treatment of a symptomatic dermatofibroma using combination therapy with fractionated CO2 laser and topical corticosteroids.