Peritoneal sarcomatosis due to undifferentiated pleomorphic sarcoma: A case report and review of the literature

Undifferentiated pleomorphic sarcoma (UPS), which was previously known as malignant fibrous histiocytoma (MFH), rarely presents in the abdomen, and sarcomatosis due to UPS has not yet been reported in the literature. Here, we present a 62-year-old man who had abdominal sarcomatosis due to UPS with a poor prognosis.

Undifferentiated pleomorphic sarcoma: prognostic factors in 42 extremity cases / Sarcoma pleomórfico indiferenciado: fatores de prognóstico em 42 casos em extremidades

ABSTRACT Introduction: Soft tissue undifferentiated pleomorphic sarcoma (UPS) in extremities is considered a rare neoplasm, corresponding to 5% of soft tissue sarcomas (STS) today. The objective was to evaluate prognostic factors related to death, local recurrence (LR), and impact on survival rates. Methods: A retrospective study including 42 patients with UPS in extremities treated surgically in a single center. Comparisons were made between demographic data, characteristics of the neoplasia, and treatment. Between the variables with statistical significance, logistic regression analysis was used. Survival rates were evaluated using Kaplan-Meier plots. To compare the effect of variables on survival rates, the Log-Rank test was used. Results: Age group of patients was from 25 to 85 years (mean 58 years), with a mean follow-up of 29.6 months. The variables with the highest effect on survival rates were sizes larger than 15 cm (T4) with p = 0.01, presence of metastatic lesions, and prognostic stage IV according to the American Joint Committee of Cancer (AJCC) with p < 0.001. The mean survival was 25.9 months. Metastasis and stage IV of AJCC were associated with a reduction in patient survival (17.8 months) with Log-Rank test p < 0.001. Conclusion: The main factors of poor prognosis related to mortality and reduction of survival of UPS in extremities were metastatic lesions and stage IV of AJCC. Level of Evidence III, Retrospective Study.

RESUMO O sarcoma pleomórfico indiferenciado (SPI) de tecidos moles em extremidades, neoplasia rara, correspondente a 5% dos sarcomas de tecidos moles (STM). Objetivo: Avaliar fatores de prognóstico relacionados a óbito, recorrência local (RL) e impacto na sobrevida geral. Métodos: Estudo retrospectivo de 42 pacientes com SPI tratados cirurgicamente em centro único. Foram comparados dados demográficos, características da neoplasia e de tratamento; e para as variáveis com significância estatística, foi realizada análise de regressão logística. A sobrevida foi avaliada através de gráficos de Kaplan-Meier; e os efeito das variáveis sobre a sobrevida, por meio do teste de log-rank. Resultados: Os pacientes tinham idades entre 25 e 85 anos (média de 58 anos), com seguimento ambulatorial médio de 29,6 meses. As variáveis com maior relação com o desfecho óbito foram tamanho maior que 15 cm (T4), com p = 0,01, metástases e estágio IV de prognóstico da American Joint Committee on Cancer (AJCC), com p < 0,001. A sobrevida média dos pacientes foi de 25,9 meses. Presença de metástase e estágio IV da AJCC foram associados à redução na sobrevida dos pacientes (17,8 meses; p < 0,001). Conclusão: Os principais fatores de mau prognóstico relacionados ao óbito e à redução da sobrevida dos pacientes com SPI foram doença metastática e estágio IV da AJCC. Nível de Evidência III, Estudo Retrospectivo.

Angiomatoid fibrous histiocytoma: Report of two cases, initially construed as sarcoma with unusual clinico-pathological features

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of uncertain differentiation with low metastatic potential, most commonly occurring in children, adolescents, and young adults, involving extremities. Due to its rare nature and diverse presentation, both clinically and morphologically, it is often misdiagnosed. It becomes important to correctly diagnose this lesion, given its distinct therapeutic implications. Here, we present the clinical, radiologic, and pathologic findings of two rare cases of AFH. Since AFH is a rare soft tissue tumor with low malignant potential, both pathologists and clinicians should be aware of this entity, when encountered with a soft tissue mass in extremities of a child or adolescent, so as to accord appropriate treatment in such cases.

Congenital solitary reticulohistiocytosis (Hashimoto - Pritzker)

Abstract Congenital and self-healing Hashimoto-Pritzker reticulohistiocytosis is the benign variant of the Langerhans cell histiocytosis (LCH) group. It is characterized by multiple skin lesions (congenital or appearing during the first days after birth), without systemic manifestations and spontaneous resolution in days to months. The authors report the case of a boy with a single congenital leg skin lesion, a rare disease variant. Through histopathology, a dense skin infiltration of S100 protein-, CD1a-, CD207-immunomarked cells was found. KI67 index was high (62%). A complete spontaneous resolution occurred 07 days after the biopsy (25 days after birth). Monolesional disease, distal limb lesion, absence of lesions in the mucous membrane or seborrheic area, and less than 25 percent of LCs with Birbeck granules were said to be possible clues for a favorable prognosis in LCs histiocytosis. But, as a precautionary measure, the child will be followed up until at least 2 years of age.

Oral benign fibrous histiocytoma in a pediatric patient: case report

Benign fibrous histiocytoma (BFH) is a common mesenchymal lesion on the skin where it is better known as dermatofibroma. It mainly affects areas of the integument exposed to the sun. It can occur in the mucous membranes, being quite unusual in the oral cavity. This article presents a rare case of benign oral fibrous histiocytoma in childhood. An eleven-year-old male patient sought the stomatology clinic of the dentistry course at a reference university in northeastern Brazil, complaining of an asymptomatic increase in volume on the cheek, with an evolution of approximately two years. On intraoral examination, a lesion in the cheek mucosa was observed, measuring approximately 4 cm. The lesion had a nodular, circumscribed, reddish appearance, showing ulcerated areas covered by a yellowish pseudomembrane on its surface. The clinical diagnostic hypothesis was pleomorphic adenoma. Excisional biopsy was performed and, after morphological and immunohistochemical evaluation, the diagnosis of BFH was reached. Although it is a lesion with an excellent prognosis, BFH must be carefully analyzed for the differential diagnosis with malignant neoplasms that may have a mild appearance, such as fibrosarcoma or dermatofibrosarcoma. In addition, other benign lesions such as desmoplastic fibroblastoma, myofibroma, myopericytoma and nodular fasciitis may, in some histological sections, mimic the microscopic appearance of benign fibrous histiocytoma. In this reported case, immunohistochemical evaluation was essential to conclude the diagnosis of BFH(AU)

Mixofibrosarcoma mandibular de bajo grado en un adulto / Low grade mandibular myxofibrosarcoma in an adult

Se describe el caso clínico de un paciente de 61 años de edad, con antecedente de buena salud, atendido en el St. Paul's Milennium Medical Hospital de Etiopía por presentar, desde hacía 2 años, una lesión en la encía marginal, con abombamiento de ambas corticales, que se extendía del primer premolar inferior derecho al izquierdo, por lo cual se decidió ingresarlo en el Servicio de Cirugía Maxilofacial para su estudio y tratamiento. Se trataba de un tumor poco frecuente y de gran tamaño, con amplia diseminación ganglionar hacia los niveles IV y V de ambas partes del cuello, de manera que se realizó el procedimiento quirúrgico. La evolución fue favorable en el posoperatorio inmediato y egresó de la institución 7 días después, pero continuó con radioterapia. Y seguimiento por consulta externa durante 24 meses después.

The clinical case of a 61-year-old patient, with a history of good health, treated at St. Paul's Milennium Medical Hospital in Ethiopia for presenting, for 2 years, a lesion in the marginal gum, with bulging of the gums, is described. both cortices, which extended from the lower right to the left first premolar, for which it was decided to admit him to the Maxillofacial Surgery Service for study and treatment. It was a rare and large tumor, with extensive lymph node dissemination to levels IV and V in both parts of the neck, so the surgical procedure was performed. The evolution was favorable in the immediate postoperative period and he was discharged from the institution 7 days later, but continued with radiotherapy. And follow-up by outpatient service for 24 months afterwards.

A case of pseudomyogenic hemangioendothelioma misdiagnosed as low-grade malignant fibrous histiocytoma and review of literature / 中南大学学报(医学版)

Pseudomyogenic hemangioendothelioma (PHE) is a rare angiogenic tumor. Histologically, the morphological characteristics of neoplastic vessels and endothelial differentiation are not obvious, and it is easy to be confused with epithelioid sarcoma, epithelioid hemangioendothelioma and myogenic tumor. PHE usually occurs in arms and legs in young people and has a significant male predominance. The tumor has a predilection for the distal extremities and its typical manifestation is multiple center invasion of a single limb, which can involve all layers of skin and subcutaneous tissues,and is often accompanied by abvious pain. Histologically, PHE is characterized by infiltrative growth of tumor. Most tumor lesions are composed of sheets and loose fascicles of plump spindle or epithelioid cells within a background of variably prominent inflammatory infiltration, which was commonly composed of neutrophils. Some cells may resemble rhabdomyoblasts, and nuclear atypia and mitosis were rare. The tumor cells generally expressed positive cytokeratin (CK), ETS-related gene (ERG), Friend leukemia virus integration 1 (FLI1) and integrase interactor 1(INI1). In some cases, the tumor cells expressed CD31. A case of a young woman was reported in this paper, who presented with a subcutaneous mass with severe pain and was chronologically misdiagnosed with herpes zoster, low-grade malignant fibrous histiocytoma and epithelioid hemangioendothelioma. In this study, the clinical and pathological features, differential diagnosis and the latest progress in therapy of PHE were analyzed based on relevant literature.

Histiocitoma fibroso maligno no tornozelo: relato de caso / Malignant fibrous histiocytoma in ankle: case report

Histiocitoma fibroso maligno (MFH) ou sarcoma pleomórfico indiferenciado (UPS) é um sarcoma moderadamente agressivo, capaz de invadir estruturas adjacentes. Trata-se de neoplasia mesenquimal que predomina em homens entre a sexta e sétima décadas de vida. Localiza-se, principalmente, nos membros inferiores, podendo acometer cabeça e pescoço, tronco e retroperitônio, com tendência à recorrência e à metástase local. O presente relato tem como objetivo apresentar um caso de MFH no tornozelo de uma mulher de 49 anos, com invasão óssea adjacente, que evoluiu com amputação transtibial. São abordados aspectos clínicos, radiológicos, histopatológicos e terapêuticos, salientando-se a importância do diagnóstico precoce.

Malignant fibrous histiocytoma (MFH) or undifferentiated pleomorphic sarcoma (UPS) is a sarcoma capable of invading adjacent structures. It is a mesenchymal neoplasia that predominates in men between the sixth and seventh decades of life. It is located mainly in the lower limbs and may affect the head and neck, trunk, and retroperitoneum, presenting a tendency to recurrence and local metastasis. This report aims to present a case of MFH in the ankle of a 49-year-old woman with an adjacent bone invasion, which evolved with transtibial amputation. Clinical, radiological, histopathological, and therapeutic aspects were addressed, highlighting the importance of early diagnosis

Immunohistochemical analysis of S100-positive epidermal Langerhans cells in dermatofibrom

Abstract Dermatofibroma is a dermal fibrohistiocytic neoplasm. The Langerhans cells are the immunocompetent cells of the epidermis, and they represent the first defense barrier of the immune system towards the environment. The objective was to immunohistologically compare the densities of S100-positive Langerhans cells in the healthy peritumoral epidermis against those in the epidermis overlying dermatofibroma (20 cases), using antibodies against the S100 molecule (the immunophenotypic hallmark of Langerhans cells). The control group (normal, healthy skin) included ten healthy age and sex-matched individuals who underwent skin biopsies for benign skin lesions. A significantly high density of Langerhans cells was observed both in the epidermis of the healthy skin (6.00 ± 0.29) and the peritumoral epidermis (6.44 ± 0.41) vs. those in the epidermis overlying the tumor (1.44 ± 0.33, p < 0.05). The quantitative deficit of Langerhans cells in the epidermis overlying dermatofibroma may be a possible factor in its development.

Multinucleate cell angiohistiocytoma: an uncommon cutaneous tumor

Abstract Multinucleate cell angiohistiocytoma is a rare, benign vascular proliferation of unknown etiology. It occurs mainly in middle-aged women and usually affects the acral regions; the lesions appear as discrete, grouped, and asymptomatic violaceous papules. Histopathology shows proliferation and dilated small vessels in the papillary dermis, fibrous stroma with thickened collagen bundles, and multinucleated giant cells. To date, there are approximately 140 cases described in the indexed literature. This report presents the case of a 62-year-old woman with a typical clinical condition, who chose not undergo treatment, considering the benign character of her illness. The clinical and immunohistological aspects of this unusual dermatological entity are emphasized.

Malignant fibrous histiocytoma - a case report

Fibrous histiocytoma is a mesenchymal neoplasm with benign and malignant varieties. This tumor mainly affects the skin of extremities in adults and may on rare occasions affect the oral cavity. The tumor has radiographic features in very rare cases. The present case report aims to conduct a clinicopathological-radiographic and immunohistochemical assessment and treatment of a patient with this lesion

Dermatoscopia do dermatofibroma aneurismático: relato de dois casos / Dermoscopy of aneurysmal dermatofibroma: report of two cases

O dermatofibroma (DF) aneurismático é um tumor benigno raro, de origem na derme, considerado uma variante de dermatofibroma. É prevalente nos membros inferiores de mulheres acima de 30 anos. Sua etiologia é desconhecida, e o exame histopatológico confirma o diagnóstico. Este trabalho apresenta dois casos de DF aneurismático, em pacientes jovens, do sexo masculino, e com lesões semelhantes: nódulo hipercrômico único, de aproximadamente 1,5cm e crescimento progressivo. O objetivo deste relato é demonstrar uma variante pouco frequente de dermatofibroma e ressaltar o possível diagnóstico diferencial com outros tumores por meio do exame dermatoscópico.

Aneurysmal dermatofibroma (DF) is a rare benign tumor originating in the dermis, considered a variant of dermatofibroma. It is more prevalent in women over 30 years of age, in the lower limbs. Its etiology is unknown and histopathological examination confirms the diagnosis. This study reports two cases of aneurysmal DF in young patients, both men, presenting similar lesions: single hyperchromic nodule, with approximately 1.5 cm and progressive growth. This report aims to demonstrate an uncommon variant of dermatofibroma and to highlight the possible differential diagnosis with other tumors through dermoscopic examination

Histopathological and clinical analysis of 150 cases of dermatofibroma / 中华皮肤科杂志

Objective To analyze histopathological and clinical features of dermatofibroma,and to explore the relationship between them.Methods Clinical and histopathological data were collected from 150 patients with histopathologically confirmed dermatofibroma in Department of Pathology,Shanghai Skin Disease Hospital from September 2017 to August 2018,and analyzed retrospectively.Results Among the 150 patients,65 were males,and 85 were females.Their age was 42 ± 13.8 years,and the course of disease ranged from 3 months to 30 years.Some of the patients had concomitant symptoms,mainly manifesting as itching,some had spontaneous pain and mild tenderness,and 18 patients had a history of injury,insect bite or infection at lesion sites.Skin lesions mainly occurred on the extremities (107 cases,71.3％),and most were solitary (105 cases,70％).Before pathological examinations,102 cases were clinically diagnosed as dermatofibroma,16 as epidermoid cyst,13 as pigmented nevus,3 as keloid,12 as skin mass,1 as malignant melanoma,1 as xanthogranuloma,1 as prurigo nodularis,and 1 as neurofibroma.Among 169 hematoxylin and eosin (HE)-stained sections,25 (14.8％) appeared to be consistent with aneurysmal dermatofibroma,66 (39.1％)with cellular dermatofibroma,36 (21.3％) with sclerosing dermatofibroma,and 22 (13.0％)with epithelioid dermatofibroma.Coexistence of two or more subtypes could be seen in 12 sections.There were also a few new variants,such as dermatofibroma with hyperplastic sweat duct (1 case),deep dermatofibroma (3 cases),dermatofibroma with epithelioid cells intermingled with hyperplastic collagen (1 case).The duration of aneurysmal dermatofibroma varied from 7 months to 30 years,and most manifested as skin masses on the lower extremities.A relatively short course of disease was observed in patients with cellular dermatofibroma,who often visited a hospital several months after the onset,and cellular dermatofibroma was commonly observed on the extremities and frequently accompanied with itching and pain.The duration of sclerosing or atrophic dermatofibroma was usually long for years or decades,and it commonly occurred on the upper limbs without concomitant symptoms.Epithelioid dermatofibroma of varied durations had various clinical manifestations,frequently occurred on the lower limbs without concomitant symptoms.Conclusions The clinical and pathological manifestations of dermatofibroma are diverse.Different dermatofibroma lesions can share similar typical histopathological manifestations,and atypical pathological features can interfere with the diagnosis of dermatofibroma.

Calcifying fibrous tumor: a clinicopathological analysis of 32 cases / 中华病理学杂志

Objective@#To investigate the clinicopathological characteristics, histogenesis, immunophenotypes, molecular genetic characteristics, diagnosis and differential diagnosis of calcifying fibrous tumors (CFT).@*Methods@#A total of 32 cases of CFT (22 cases from Henan Provincial People′s Hospital and 10 cases from PLA Army Medical Center) diagnosed between June 2009 and February 2019 were reviewed. The clinical and pathologic data were analyzed.@*Results@#There were 12 male and 20 female patients, aged from 15 to 63 years (mean 40.8 years). Eleven cases occurred in stomach, four cases in retroperitoneum, four cases in ovary, two cases in scrotum, two cases in mediastinum, two cases in head and neck, one case each in thoracic cavity, lung, adrenal gland, kidney, sigmoid colon, epididymis and mesosalpinx. All the tumors were solid masses with clear boundaries. The maximal dimension of the tumors ranged from 0.6 to 10.0 cm. Microscopically, there was hypocellular stromal sclerosis and wavy storiform coarse collagen with superimposed scattered or patchy lymphocytes and plasma cells; calcification or gravel formation were also detected. Immunohistochemistry showed that spindle cells were positive for vimentin and some were positive for CD34; and they were negative for calponin, SMA, desmin, S-100 protein, SOX10, STAT6, β-catenin, ALK, CD117, DOG1, CKpan, and EMA. No ALK rearrangement was detected by FISH in all cases. No C-KIT and PDGFRA mutation was detected in all the tested 11 cases of stomach, four cases of retroperitoneal and one case of sigmoid colon CFT. MDM2 was not amplified by FISH in all four tested cases of retroperitoneal CFT.@*Conclusions@#CFT is a rare benign tumor of fibroblastic cell origin. The diagnosis mainly depends on histomorphologic analysis and immunophenotyping. CFT should be differentiated from other benign and malignant spindle cell mesenchymal tumors.

Clinicopathologic and molecular characteristics of myxoid angiomatoid fibrous histiocytoma / 中华肿瘤杂志

Objective@#To investigate the molecular genetic and clinicopathologic characteristics, immunophenotypes, diagnostic and differential diagnostic features of myxoid angiomatoid fibrous histiocytoma (MAFH).@*Methods@#Three cases of MAFH were collected from the archives of Zhejiang Provincial People′s Hospital between January 2015 to August 2018. The clinical and radiologic features, histomorphology, immunohistochemistry, molecular genetics and prognosis were analyzed.@*Results@#Patients consisted of 2 women and 1 man aged 37 years, 46 years, and 57 years, respectively. The clinical manifestations of 3 patients were presented as a painless, slowly-enlarged mass with a duration ranging of 2 weeks, 2 months and 50 years. These tumors were located at the deep somatic soft tissue of extremities or limbs (right hip, left forearm, left wrist, respectively) and 2 were preoperatively considered as ganglion cyst or giant cell tumor of tendon sheath by imaging examinations. The diameter of circumscribed mass lesion was ranged from 3.0 to 7.5 cm, which exhibited a gray white to tan and gelatinous cut surface. Extensive hemorrhage and cystic changes were observed in 2 cases. Under low magnification, all tumors showed a dense fibrous pseudo-capsule with a peritumoral lymphoplasmacytic cuff and a multi-nodular growth pattern. Blood-filled cystic spaces were observed in 2 tumors. The myxoid stroma occupied 60.0%, 80.0% and 90.0% area of the entire tumor, respectively. Within the myxoid areas, tumor cells were oval to stellate and arranged in cord-like, microcystic and reticular growth patterns. Transitions of myxoid tumor components to more solid areas with typical histology of angiomatoid fibrous histiocytoma (AFH) were observed at least focally in all the three cases. The tumor cells exhibited minimal atypia and scarce mitoses (1 to 2/50 HPF) without necrosis, and prominently focal intracytoplasmic vacuoles were identified in one case. The results of immunohistochemistry staining showed that, 2/3 cases focally expressed desmin, 2/3 focally expressed epithelial membrane antigen (EMA), and 1/3 focally expressed CD99. The positive index of Ki67 was approximately 1% to 5%. Fluorescence in situ hybridization analysis showed that EWSR1 gene rearrangement occurred in all of the three cases. During the period of follow-up, one case showed local recurrence at 15 months, one case showed postoperative recurrence at 24 months, and the recurrent tumor slowly grew for 120 months until the second resection, without recurrence at the following 2 months. The left case showed a disease-free survival at 32 months.@*Conclusions@#MAFH is a rare subtype of AFH with a low-grade behavior and may lead to diagnostic confusions. Carefully searching for the typical AFH histomorphology and combining with EWSR1 gene rearrangement test can help to distinguish MAFH from other mimickers.

First report of hemosiderotic adenodermatofibroma in Brazil / Primeiro relato de adenodermatofibroma hemossiderótico no Brasil

ABSTRACT Hemosiderotic adenodermatofibroma is a recently recognized lesion, characterized by a dermal nodule with cystic structures of an apocrine gland, surrounded by a dermatofibroma-like stroma with hemosiderotic macrophages. We present the first case report of this entity in Brazil together with representative images, in addition to a review on the subject and discussion about the apocrine origin of this lesion.

RESUMEN El dermatoadenofibroma hemosiderótico es una lesión recientemente descrita, caracterizada por un nódulo dérmico con estructuras quísticas de una glándula apocrina, rodeado por un estroma del tipo dermatofibroma con macrófagos hemosideróticos. Presentamos el primer reporte de caso de esa entidad en Brasil, junto a imágenes representativas, además de una revisión del tema y discusiones acerca del origen apocrina de esa lesión.

RESUMO Adenodermatofibroma hemossiderótico é uma lesão recentemente conhecida, caracterizada por um nódulo dérmico com estruturas císticas de uma glândula apócrina, circundado por um estroma do tipo dermatofibroma com macrófagos hemossideróticos. Apresentamos o primeiro relato de caso dessa entidade no Brasil junto a imagens representativas, além de uma revisão do assunto e discussões sobre a origem apócrina dessa lesão.

Calcifying fibrous tumor: a clinicopathological analysis of 32 cases / 中华病理学杂志

To investigate the clinicopathological characteristics, histogenesis, immunophenotypes, molecular genetic characteristics, diagnosis and differential diagnosis of calcifying fibrous tumors (CFT). A total of 32 cases of CFT (22 cases from Henan Provincial People's Hospital and 10 cases from PLA Army Medical Center) diagnosed between June 2009 and February 2019 were reviewed. The clinical and pathologic data were analyzed. There were 12 male and 20 female patients, aged from 15 to 63 years (mean 40.8 years). Eleven cases occurred in stomach, four cases in retroperitoneum, four cases in ovary, two cases in scrotum, two cases in mediastinum, two cases in head and neck, one case each in thoracic cavity, lung, adrenal gland, kidney, sigmoid colon, epididymis and mesosalpinx. All the tumors were solid masses with clear boundaries. The maximal dimension of the tumors ranged from 0.6 to 10.0 cm. Microscopically, there was hypocellular stromal sclerosis and wavy storiform coarse collagen with superimposed scattered or patchy lymphocytes and plasma cells; calcification or gravel formation were also detected. Immunohistochemistry showed that spindle cells were positive for vimentin and some were positive for CD34; and they were negative for calponin, SMA, desmin, S-100 protein, SOX10, STAT6, β-catenin, ALK, CD117, DOG1, CKpan, and EMA. No ALK rearrangement was detected by FISH in all cases. No C-KIT and PDGFRA mutation was detected in all the tested 11 cases of stomach, four cases of retroperitoneal and one case of sigmoid colon CFT. MDM2 was not amplified by FISH in all four tested cases of retroperitoneal CFT. CFT is a rare benign tumor of fibroblastic cell origin. The diagnosis mainly depends on histomorphologic analysis and immunophenotyping. CFT should be differentiated from other benign and malignant spindle cell mesenchymal tumors.

A rare case of benign fibrous histiocytoma of the cornea

Fibrous histiocytoma (FH) commonly occurs in the superficial layers of the skin. Orbit and limbus are documented ophthalmic sites of involvement but isolated corneal FH has never been reported in literature. We present the first case of FH exclusively involving the cornea where a 10-year-old male child presented with a 3-month history of a painless growth on the superior cornea of the right eye with deterioration of vision. Tumor excision with therapeutic penetrating keratoplasty was done and the histopathological examination confirmed the diagnosis. There was no recurrence and the corneal graft was clear at 1 year.

Aneurysmal fibrous histiocytoma

Aneurysmal fibrous histiocytoma is rare clinicopathological variant of Cutaneous Fibrous Histiocytoma. The clinical manifestation of Aneurysmal Fibrous Histiocytoma is often confusing to distinguish from other skin lesions. Most of the cases showed rapid increase in size or a history of recurrence, however histologically all are almost similar. Rarity of aneurysmal fibrous histiocytoma and high numbers of recurrence rate poses a big diagnostic challenge. Late treatment will result in a decrease until loss of function of the affected region.  In this article, author reported the case of aneurysmal fibrous histiocytoma of the hand in 7 years old girl with restricted at 2nd-3rd metacarpophalangeal joints. The patient had undergone a series of investigations until finally a wide excision was carried out. Excision tissue was performed CD 68 and CD 34  immunocytochemical smear to establish the diagnosis. It was not simple to make diagnosis aneurysmal fibrous histiocytoma. While it is benign, the lesion can appear malignant, and one should consider an excisional biopsy to rule out malignant conditions. The diagnosis had to be confirmed by histopathological and performed immunocytochemical smear. It was often necessary to take aggressive actions with wide excision and reconstruction.

Fibriohistiocitoma en fosa infratemporal derecha. Reporte de un caso / Right infratemporal fossa Fibriohistiocytoma. Case Report

RESUMEN Fundamento: El fibriohistiocitoma benigno es infrecuente que se presente en la adolescencia y menos con la ubicación topográfica inusual en fosa infratemporal derecha. Objetivo: Ilustrar la presentación de un fibriohistiocitoma benigno en fosa infratemporal derecha en un paciente masculino de 17 años de edad. Presentación de caso: Se reportó un caso en un paciente masculino de 17 años de edad, con un fibriohistiocitoma que tiene un comportamiento biológico benigno y se localiza en fosa infratemporal derecha; bajo el músculo temporal, en el servicio de Cirugía Maxilofacial de Sancti Spíritus. Conclusiones: El fibrohistiocitoma puede aparecer en cualquier parte del cuerpo es poco frecuente en cabeza y cuello, menos aún en fosa infratemporal, como se reportó en este caso.

ABSTRACT Background: Benign fibriohistiocytoma is uncommon to occur in adolescence and less with the unusual topographic location in the right infratemporal fossa. Objective: To illustrate the presentation of a benign fibriohistiocytoma in the right infratemporal fossa in a 17-year-old male patient. Case presentation: A case was reported in a 17-year-old male patient, with a fibriohistiocytoma that has a benign biological behavior and is located in the right infratemporal fossa; under the temporal muscle, in the Maxillofacial Surgery service of Sancti Spíritus. Conclusions: Fibrohistiocytoma can appear in any part of the body, it is rare in the head and neck, even less in the infratemporal fossa, as reported in this case.