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.

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

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.

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.

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

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.

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.

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.

Scanning electron microscopy of dermatofibroma

Abstract: Dermatofibroma is a proliferation of spindle cells located in the dermis. We used scanning electron microscopy to examine two histologically confirmed lesions and observed preserved collagen bundles in the perilesional area. In the lesional area, the collagen was denser, without formation of bundles. Higher magnification showed collagen with mesh-like appearance similar to stretched tufts of cotton. Very high magnification evidenced the tufts of cotton and spindle cells measuring 2 to 12 microns.

Myxoid variant of angiomatoid fibrous histiocytoma: a clinicopathologic analysis of 3 cases / 中华病理学杂志

Objective@#To study clinicopathologic features, diagnosis and differential diagnosis of myxoid variant of angiomatoid fibrous histiocytoma (AFH).@*Methods@#Three cases of myxoid variant of AFHs were collected from First Affiliated Hospital of Nanjing Medical University during 2008 and 2017. EnVision method and fluorescence in situ hybridization(FISH) were used to detect immunophenotype and EWSR1 gene rearrangement, respectively.@*Results@#There were 2 males and l female with age at 13, 31, and 42 years, respectively. The patients presented with a painless mass located superficially (subcutaneous or submucosal) in two cases or deep-seated (retroperitoneum) in one case. Grossly, the diameters of tumors were 1, 7, and 2 cm, respectively. The cut surface was solid and firm, tan to gray in colour. Histologically, the circumscribed tumor had fibrous pseudocapsule and peritumoal lymphoplasmacytic infiltrates. The tumor cells arranged in vaguely nodular growth pattern, with prominent myxoid stroma (present in 60% to 100% of the entire tumor). In hypocellular myxoid areas, the spindle to stellate tumor cells arranged in cords or reticular pattern, or in a haphazard manner. However, histiocytoid cells arranged in fascicular, sheet-like, or whorled growth pattern, as in classical AFH, were also identified in hypercelluar areas. Mild to moderate atypia was observed with low mitotic rate of (0-2)/10 HPF. Tumor necrosis was not seen. One case presented with slit-like hemorrhage and sclerosing collagen intermingled with myxoid matrix was identified in 1 case. Immunohistochemically, all cases were positive for CD68 and CD163. Two of three were positive for desmin, EMA, CD99 and one for Calponin, SMA. All cases were negative for S-100 protein, CD34, CD31, CD35, CD21 and CKpan. FISH detection was positive for EWSRl gene in all cases. Available clinical follow-up was obtained in 2 cases, revealing no evidence of disease in 6 and 89 months, respectively.@*Conclusions@#Myxoid variant of AFH is a histological subtype of AFH, with clinical features, immunophenotypes, genomic profiles and biological behavior similar to typical AFH. Their unusual morphology is easily confused with a variety of other myxoid mesenchymal neoplasms, including myoepithelioma and nerve sheath tumors.

Comments to "Deep Benign Fibrous Histiocytoma Showing Multiple Metastases" / 대한피부과학회지

No abstract available.

Hemosiderotic dermatofibroma

Abstract: We report a rare clinical case of hemosiderotic dermatofibroma in a 36-year-old female patient. The main dermatoscopic finding was represented by homogeneous blue-gray pigmentation. The aim of this report is to demonstrate the rarity of the lesion and the dermatoscopic importance it assumes by sharing a blue-gray homogeneous pattern with other benign and malignant lesions.

Deep Benign Fibrous Histiocytoma Showing Multiple Metastases / 대한피부과학회지

Some morphological variants of benign fibrous histiocytoma (dermatofibroma) present with distinct clinical features. In particular, atypical, aneurysmal, and cellular fibrous histiocytoma are associated with a significant risk of local recurrence. Furthermore, very rarely, cutaneous fibrous histiocytoma can lead to metastatic disease. Deep benign fibrous histiocytoma is an uncommon and poorly recognized clinical subtype that arises in subcutaneous or deep soft tissue. Deep benign fibrous histiocytoma has many histologic features in common with cellular fibrous histiocytoma. Deep benign fibrous histiocytoma recurs in approximately 20% of cases and may rarely metastasize. We report a rare case of deep benign fibrous histiocytoma with metastatic potential in a 38-year-old man who presented with a 2-year history of a recurrent hard mass on his left shoulder.

Benign fibrous histiocytoma of the tongue: a case report / Histiocitoma fibroso benigno em língua: relato de caso

The fibrous histiocytoma is a soft tissue neoplasm that affects the dermis and the subcutaneous tissue, rarely is found in the oral cavity and perioral regions, and is originated from the proliferation of fibroblasts and histiocytes. The objective of this paper is to report a case of Benign Fibrous Histiocytoma in a 30-year-old male patient, complaining of a painless nodule in the tongue for about six months. With diagnostic clinical hypotheses of Fibrous Hyperplasia, Neurofibroma, Traumatic Neuroma, Fibrous Histiocytoma, Granular Cell Tumor or Ectomesenchymal Chondromyxoid Tumor a excisional biopsy was performed. The histopathological examination revealed a non-encapsulated proliferation of spindle cells with some giant multinucleated cells in the periphery of the lesion. Immunohistochemical reactions were performed, staining only for vimentin in the spindle cells and for CD68 in the multinucleated giant cells. According to these characteristics, the final diagnosis was Benign Fibrous Histiocytoma. The correct diagnosis of spindle shaped cell neoplasia must be performed with the aid of histopathological analysis and immunohistochemistry, mainly because the morphological similarities with other benign and malignant lesions. (AU)

O Histiocitoma Fibroso é uma neoplasia de partes moles que acomete a derme e o tecido subcutâneo, raramente é encontrado na cavidade oral e regiões periorais, e tem origem a partir da proliferação de fibroblastos ou histiócitos. O objetivo deste artigo é relatar um caso de Histiocitoma Fibroso Benigno em um paciente masculino, 30 anos de idade, com um nódulo indolor, bem delimitado, com duração de cerca de seis meses, localizado no dorso anterior da língua. Com as hipóteses clínicas diagnósticas de Hiperplasia Fibrosa, Neurofibroma, Neuroma Traumático, Histiocitoma Fibroso, Tumor de Células Granulares e Tumor Condromixoide Ectomesenquimal uma biópsia foi realizada sob anestesia local e a lesão foi fixada em formol a 10% e enviada para análise histopatológica. O exame histopatológico revelou uma proliferação não-encapsulada de células fusiformes com algumas células gigantes multinucleadas na periferia da lesão. A marcação imunohistoquímica foi positiva para CD68 nas células gigantes multinucleadas e para vimentina nas células fusiformes. O diagnóstico final foi de Histiocitoma Fibroso Benigno. Para um diagnóstico correto, este deve ser feito correlacionando características clínicas, análise histopatológica e imunohistoquímica devido à similaridade microscópica do Histiocitoma Fibroso com outras lesões com aspecto fusocelular, assim como similaridade clínica com outras lesões benignas e malignas.(AU)

Dermatofibroma simulating seborrheic keratosis dermoscopically

Abstract: Dermatofibroma is a frequent benign tumor of easy clinical diagnosis in most cases, but that can mimic other dermatoses. Dermoscopy may help to define the diagnosis and its classical pattern is a central white area, similar to a scar, surrounded by a discrete pigment network. However, dermoscopic findings are not always typical. We describe here a case of dermatofibroma exhibiting ridges, furrows and pseudocomedos, a pattern which is typical of seborrheic keratosis, in dermoscopy.

Benign Fibrous Histiocytoma with Cystic Change of the Femur: a Case Report

Benign fibrous histiocytoma (BFH) is a rare benign primary skeletal tumor that occurs commonly in the long bones, spine and pelvis. BFH constitutes a diagnostic challenge because it shares clinical background, radiological characteristics, and histological features with other fibrous lesions such as non-ossifying fibroma, giant cell tumor. We present a case of BFH with cystic change that occurred in the distal femur. We did not identify any case of BFH with cystic change involving the majority of the lesion that occurred in the metaepiphysis of the long bone.

Radiological and clinicopathological analysis of benign fibrous histiocytoma of bone / 实用医学杂志

Objective To explore the imaging radiological and clinicopathological characteristics of benign fibrous histiocytoma (BFH) of bone, in order to improve the imaging diagnostic accuracy of the disease Methods Radiological and clinical data of 16 patients with BFH confirmedbypathology were retrospectively analyzed , and relevant literatures were reviewed. Results The X-ray findings of 14 BFHpatients were osteolytic lesion , expansive destruction of bone,without soft tissue involvement, 10 with residual apophysis,9 with osteosclerosis marginal; CT was performed on 2 patients. Expansive destruction of bone and soft tissue densities were seen inside the loops , 1 case remarkable enhancement of the lesion. MRI was performed on 9 patients and 5 cases showed long T1 and T2 signal intensity; 1case showed equal T1 and long T2 signal intensity in cystic part and equal T1 and T2 signal intensity in the solid part. One patient had pathological fracture, and the surrounding soft tissue invaded, showing equal T1 and long T2 signal intensity. Two lesions located at spinous process of cervical vertebra , showing long T1 short T2 and equal T1 and T2 signal intensity. Linear low signal separation shadow in T1 and T2 could be seen in 6 cases. Long T1 and short T2 sclerotic ring were seen in 4 cases. Solid part of tumor and linear separator were obviously enhanced. Conclusions The radiological findings of benign fibrous histioeytoma of bone indicate some characteristics. The combination with X-ray , CT and MRI examination is helpful to improve the diagnostic accuracy of the disease before operation.

Fibrohistiocitoma profundo benigno peritoneal en un niño de 14 meses de edad. Reporte del caso / Deep benign fibrous peritoneal histiocytoma in a 14 month-old child. Case report / Fibrohistiocitoma profundo benigno peritoneal numa criança de 14 meses de idade. Reporte do caso

El fibrohistiocitoma benigno es un tumor raro en niños y más frecuente en adultos, que afecta principalmente la piel de las extremidades; su tamaño es usualmente pequeño. Según datos de la Organización Mundial de la Salud, menos del 1 % de los fibrohistiocitomas benignos son profundos. No se ha informado previamente su presencia en el peritoneo de niños. Por su gran interés, presentamos el caso de un niño de 14 meses con fibrohistiocitoma benigno peritoneal irresecable.

Benign fibrous histiocytoma is very rare in children, and more frequent in adults. It is commonly found in the skin, especially in the limbs. Usually it is small. According to the World Health Organization, less than 1 % of benign fibrous histiocytomas are found in deep tissues. Peritoneal location of this kind of tumor has not been previously reported in children. Due to its great interest, we report the case of a 14 month-old male with a peritoneal benign fibrous histiocytoma. No resection of the lesion was feasible.

O fibrohistiocitoma benigno é raro em crianças e mais frequente em adultos. Afeta mais a pele das extremidades e seu tamanho é pequeno. Segundo dados da Organização Mundial da Saúde, menos de 1 % dos fibrohistiocitomas benignos são profundos. Não se informou previamente sua presença no peritônio de crianças. Por seu grande interesse, apresentamos o caso de uma criança de 14 meses com fibrohistiocitoma benigno peritoneal irressecável.

Oral Benign Fibrous Histiocytoma of Chin: A Rare Case Report and Literature Review.

Fibrous histiocytoma is a benign soft tissue neoplasm that may present as a fi brous mass any-where in the human body. Th e involvement of the oral cavity is extremely rare and very few cases have been reported in the literature until date. We here report a case of benign fi brous histiocytoma localized in the oral cavity in mandibular chin region and the treatment approach that need to be followed for such cases. Th e clinical and histological features of the lesion are discussed precisely in the light of a literature review of this pathology occurring in soft tissues in the head and neck region.