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2.
J Cancer Res Ther ; 16(6): 1524-1527, 2020.
Article En | MEDLINE | ID: mdl-33342824

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.


Chemoradiotherapy, Adjuvant/adverse effects , Li-Fraumeni Syndrome/therapy , Neoplasms, Radiation-Induced/radiotherapy , Proton Therapy , Re-Irradiation/methods , Child , Female , Histiocytoma, Benign Fibrous/diagnosis , Histiocytoma, Benign Fibrous/genetics , Histiocytoma, Benign Fibrous/therapy , Humans , Li-Fraumeni Syndrome/diagnosis , Li-Fraumeni Syndrome/genetics , Magnetic Resonance Imaging , Neoplasm Recurrence, Local/diagnosis , Neoplasm Recurrence, Local/genetics , Neoplasm Recurrence, Local/therapy , Neoplasms, Radiation-Induced/diagnosis , Osteosarcoma/diagnosis , Osteosarcoma/therapy , Positron-Emission Tomography , Rhabdomyosarcoma/therapy , Treatment Outcome
3.
Actas dermo-sifiliogr. (Ed. impr.) ; 111(3): 257-260, abr. 2020. ilus
Article Es | IBECS | ID: ibc-191530

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


Humans , Female , Adult , Middle Aged , Histiocytoma, Benign Fibrous/therapy , Lasers, Dye/therapeutic use , Intense Pulsed Light Therapy , Dermoscopy/methods , Lasers, Solid-State/therapeutic use , Prospective Studies , Patient Satisfaction , Hypopigmentation/diagnosis
5.
Australas J Dermatol ; 61(1): e22-e27, 2020 Feb.
Article En | MEDLINE | ID: mdl-31264202

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.


Head and Neck Neoplasms/epidemiology , Head and Neck Neoplasms/pathology , Histiocytoma, Benign Fibrous/epidemiology , Histiocytoma, Benign Fibrous/pathology , Skin Neoplasms/epidemiology , Skin Neoplasms/pathology , Aged , Aged, 80 and over , Female , Head and Neck Neoplasms/therapy , Histiocytoma, Benign Fibrous/therapy , Humans , Incidence , Male , Middle Aged , Retrospective Studies , Skin Neoplasms/therapy , Spain
7.
Rom J Ophthalmol ; 63(3): 273-276, 2019.
Article En | MEDLINE | ID: mdl-31687631

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.


Conjunctiva/pathology , Conjunctival Diseases/diagnosis , Cryotherapy/methods , Histiocytoma, Benign Fibrous/diagnosis , Ophthalmologic Surgical Procedures/methods , Conjunctival Diseases/therapy , Diagnosis, Differential , Histiocytoma, Benign Fibrous/therapy , Humans , Male , Middle Aged , Slit Lamp
9.
J Med Imaging Radiat Oncol ; 60(5): 693-695, 2016 Oct.
Article En | MEDLINE | ID: mdl-27302690

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.


Histiocytoma, Benign Fibrous/therapy , Pulmonary Sclerosing Hemangioma/therapy , Radiotherapy , Humans , Treatment Outcome
10.
J Obstet Gynaecol Res ; 42(8): 1021-5, 2016 Aug.
Article En | MEDLINE | ID: mdl-27080826

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.


Histiocytoma, Benign Fibrous/diagnostic imaging , Histiocytoma, Benign Fibrous/therapy , Pregnancy Complications/surgery , Splenectomy , Splenic Neoplasms/diagnostic imaging , Splenic Neoplasms/surgery , Adult , Female , Gestational Age , Histiocytoma, Benign Fibrous/complications , Histiocytoma, Benign Fibrous/pathology , Humans , Magnetic Resonance Imaging , Pregnancy , Pregnancy Outcome , Pruritus/complications , Splenic Neoplasms/complications , Splenic Neoplasms/pathology , Ultrasonography
11.
Rev. patol. respir ; 19(1): 22-25, ene.-mar. 2016. ilus, tab
Article Es | IBECS | ID: ibc-150271

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


Humans , Female , Aged , Histiocytoma, Benign Fibrous/diagnosis , Histiocytoma, Benign Fibrous/etiology , Histiocytoma, Benign Fibrous/therapy , Tuberculosis, Pulmonary , Histiocytosis, Langerhans-Cell/etiology , Histiocytosis, Langerhans-Cell/pathology , Immunohistochemistry/methods , Immunohistochemistry , Biopsy/methods , Biopsy , Case Reports
14.
Anticancer Res ; 35(11): 5717-35, 2015 Nov.
Article En | MEDLINE | ID: mdl-26503993

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.


Biomarkers, Tumor/metabolism , Fibroma/diagnosis , Histiocytoma, Benign Fibrous/diagnosis , Skin Neoplasms/diagnosis , Xanthomatosis/diagnosis , Fibroma/metabolism , Fibroma/therapy , Histiocytoma, Benign Fibrous/metabolism , Histiocytoma, Benign Fibrous/therapy , Humans , Prognosis , Skin Neoplasms/metabolism , Skin Neoplasms/therapy , Xanthomatosis/metabolism , Xanthomatosis/therapy
16.
Medicine (Baltimore) ; 94(3): e369, 2015 Jan.
Article En | MEDLINE | ID: mdl-25621678

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.


Bone Neoplasms/diagnostic imaging , Bone Neoplasms/pathology , Cell Transformation, Neoplastic/pathology , Fibrous Dysplasia, Monostotic/diagnostic imaging , Fibrous Dysplasia, Monostotic/pathology , Magnetic Resonance Imaging , Tomography, X-Ray Computed , Adolescent , Adult , Aged , Bone Neoplasms/therapy , Combined Modality Therapy , Female , Femur/diagnostic imaging , Femur/pathology , Fibrosarcoma/diagnostic imaging , Fibrosarcoma/pathology , Fibrosarcoma/therapy , Fibrous Dysplasia, Monostotic/diagnosis , Histiocytoma, Benign Fibrous/diagnostic imaging , Histiocytoma, Benign Fibrous/pathology , Histiocytoma, Benign Fibrous/therapy , Humans , Humerus/diagnostic imaging , Humerus/pathology , Ilium/diagnostic imaging , Ilium/pathology , Male , Middle Aged , Osteosarcoma/diagnostic imaging , Osteosarcoma/pathology , Osteosarcoma/therapy , Prognosis , Retrospective Studies , Tibia/diagnostic imaging , Tibia/pathology , Young Adult
17.
J. vasc. bras ; 13(3): 249-253, Jul-Sep/2014. graf
Article En | LILACS | ID: lil-727125

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...


Humans , Female , Adolescent , Histiocytoma, Benign Fibrous/diagnosis , Histiocytoma, Benign Fibrous/therapy , Histiocytoma, Benign Fibrous , Mouth , Sclerotherapy/nursing , Lip/pathology
18.
Arch Ital Urol Androl ; 86(2): 158-9, 2014 Jun 30.
Article En | MEDLINE | ID: mdl-25017607

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%.


Histiocytoma, Benign Fibrous , Rare Diseases , Urinary Bladder Neoplasms , Fatal Outcome , Histiocytoma, Benign Fibrous/diagnosis , Histiocytoma, Benign Fibrous/therapy , Humans , Male , Middle Aged , Rare Diseases/diagnosis , Rare Diseases/therapy , Urinary Bladder Neoplasms/diagnosis , Urinary Bladder Neoplasms/therapy
19.
Musculoskelet Surg ; 98(3): 251-4, 2014 Dec.
Article En | MEDLINE | ID: mdl-23263835

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.


Bone Neoplasms/diagnostic imaging , Femur Neck/diagnostic imaging , Fibroma/diagnostic imaging , Histiocytoma, Benign Fibrous/diagnostic imaging , Joint Capsule/diagnostic imaging , Bone Neoplasms/pathology , Bone Neoplasms/surgery , Bone Neoplasms/therapy , Bone Transplantation , Conservative Treatment , Curettage , Diagnosis, Differential , Femur Neck/pathology , Femur Neck/surgery , Fibroma/pathology , Fibroma/surgery , Fibroma/therapy , Histiocytoma, Benign Fibrous/pathology , Histiocytoma, Benign Fibrous/surgery , Histiocytoma, Benign Fibrous/therapy , Humans , Joint Capsule/pathology , Joint Capsule/surgery , Young Adult
20.
J Drugs Dermatol ; 12(12): 1483-4, 2013 Dec.
Article En | MEDLINE | ID: mdl-24301252

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.


Glucocorticoids/therapeutic use , Histiocytoma, Benign Fibrous/therapy , Lasers, Gas/therapeutic use , Administration, Cutaneous , Combined Modality Therapy , Dermatologic Agents/administration & dosage , Dermatologic Agents/therapeutic use , Female , Follow-Up Studies , Glucocorticoids/administration & dosage , Histiocytoma, Benign Fibrous/pathology , Humans , Middle Aged , Treatment Outcome
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