ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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)
Subject(s)
Humans , Male , Child , Histiocytoma, Benign Fibrous/diagnosis , Histiocytoma, Benign FibrousABSTRACT
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.
Subject(s)
Adolescent , Adult , Child , Female , Humans , Male , Biomarkers, Tumor , Diagnosis, Differential , Diagnostic Errors , Hemangioendothelioma, Epithelioid/pathology , Hemangioma , Histiocytoma, Malignant Fibrous/diagnosis , Pain , Precancerous Conditions/diagnosisABSTRACT
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
ABSTRACT
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
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
Abstract We are going to present a case of malignant fibrous histiocytoma in the right atrium, which is a very rare entity. The patient had a right atrial mass, which prolapsed through the tricuspid valve into the right ventricle, causing functional tricuspid valve stenosis. The tumor was completely resected and the patient had an uneventful postoperative period. Histopathological examination reported malignant fibrous histiocytoma. The patient presented to the emergency department five weeks after discharge with dyspnea and palpitation. Echocardiography and magnetic resonance imaging revealed recurrent right atrial tumor mass. His clinical status has worsened, with syncope and acute renal failure. On the repeated echocardiography, suspected tumor recurrence was observed in left atrium, which probably caused systemic embolization. Considering the aggressive nature of the tumor and systemic involvement, our Heart Council decided to provide palliative treatment by nonsurgical management. His status deteriorated for the next few days and the patient succumbed to a cardiac arrest on the 4th day.
Subject(s)
Humans , Male , Histiocytoma, Malignant Fibrous/pathology , Heart Neoplasms/pathology , Magnetic Resonance Imaging , Echocardiography , Tomography, X-Ray Computed , Tricuspid Valve Prolapse/pathology , Tricuspid Valve Prolapse/diagnostic imaging , Coronary Angiography , Fatal Outcome , Rare Diseases , Histiocytoma, Malignant Fibrous/diagnostic imaging , Heart Atria/pathology , Heart Atria/diagnostic imaging , Heart Neoplasms/diagnostic imaging , Neoplasm Recurrence, LocalABSTRACT
Primary malignant fibrous histiocytoma (MFH) of the chest wall is extremely rare and is characterized by aggressive features, including a high incidence of local recurrence and distant metastasis. Surgical resection of the chest wall is the primary modality of management. However, surgical treatment is not generally recommended in patients with evidence of distant metastasis. Here, we present a case of chest wall MFH along with a schwannoma mimicking distant metastasis in the right upper arm. The patient was treated by radical en bloc resection and survived for more than 9 years without recurrence.
Subject(s)
Humans , Arm , Histiocytoma, Malignant Fibrous , Incidence , Neoplasm Metastasis , Neurilemmoma , Positron-Emission Tomography , Recurrence , Thoracic Wall , ThoraxABSTRACT
An undifferentiated pleomorphic sarcoma (UPS), also known as a malignant fibrous histiocytoma in the past, commonly involves the soft tissue of the extremities and the retroperitoneum. However, a primary UPS of the colon mesentery is very rare. A 69-year-old male patient visited our outpatient department for treatment of an enlarged, palpable mass in the right lower quadrant (RLQ). Computed tomography showed a 15-cm multilobulated, heterogeneous, enhanced mass in the RLQ, which we suspected originated from the colon. He underwent a right hemicolectomy, and the pathologic result was a colon mesenteric UPS. We report a rare case of a primary UPS of the colon mesentery and discuss the characteristics of this neoplasm in reference to the literature.
Subject(s)
Aged , Humans , Male , Colon , Extremities , Histiocytoma, Malignant Fibrous , Mesentery , Outpatients , SarcomaABSTRACT
Abstract Introduction Benign fibrous histiocytomas are common lesions of the skin that rarely affect the tongue. Such cases are available in the literature exclusively as case reports. Similarly, malignant fibrous histiocytoma, now classified as undifferentiated pleomorphic sarcoma, is exceedingly rare in the tongue and not fully understood. Objectives This study systematically reviews the available literature discussing the clinical and pathological features of malignant and benign fibrous histiocytomas. Data Synthesis A total of 20 cases were included in this review. Patient-level data were extracted from cases to include clinical presentation, workup, treatment, and outcome. Conclusion Benign fibrous histiocytomas are consistent in clinical and histopathologic presentation. Surgical treatment provides excellent outcome, with no recurrence in all excised cases. Malignant tumors have a more aggressive clinical and pathological presentation. Surgical treatment with possible adjuvant radiotherapy resulted in recurrence in 40% of cases (follow-up of 24 months), and death due to disease in 47% of patients (follow-up of 19 months).
ABSTRACT
No abstract available.
Subject(s)
Epithelioid Cells , Granuloma, Pyogenic , Histiocytoma , Histiocytoma, Benign FibrousABSTRACT
Myxofibrosarcoma is the most common soft tissue sarcoma that occurs in late adult life, mainly occurring in the lower extremities and trunk. However, head and neck myxofibrosarcoma is extremely rare. The most reliable treatment of adult soft tissue sarcoma is surgical resection with negative margin. A 79-year-old man presented with a left cheek mass first detected six months ago. The pathologic report of the mass showed that it was myxofibrosarcoma and consequently postoperative radiotherapy was done. However, distant and locoregional metastasis occurred postoperatively. We report this case with a brief review of literature.
Subject(s)
Adult , Aged , Humans , Cheek , Head , Histiocytoma, Malignant Fibrous , Lower Extremity , Myxosarcoma , Neck , Neoplasm Metastasis , Radiotherapy , SarcomaABSTRACT
El histiocitoma fibroso maligno es el sarcoma de partes blandas más frecuente de la adultez tardía. Se desarrolla habitualmente en los miembros inferiores y en el retroperitoneo. Su pronóstico es malo, la recidiva local y las metástasis a distancia. Se presenta el caso de un paciente con metástasis en el estómago de un histiocitoma fibroso maligno. El objetivo de esta publicación es la presentación de una forma poco común de diseminación metastásica del histiocitoma fibroso maligno(AU)
Malignant fibrous histiocytoma is the most common soft tissue sarcoma of late adult life. It usually develops in the lower extremities and in the retroperitonium. Prognosis is fairly poor; local relapse and distant metastasis are the common natural history. The is presented of a patient with stomach metastasis of malignant fibrous histiocytoma. This paper is aimed at presenting a rare way of metastatic dissemination of the malignant fibrous histiocytoma(AU)
Subject(s)
Humans , Male , Adult , Histiocytoma, Malignant Fibrous/diagnosis , Neoplasm Metastasis , Stomach Neoplasms/drug therapy , Tomography, X-Ray Computed/methodsABSTRACT
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.
Subject(s)
Humans , Female , Adult , Skin Neoplasms/pathology , Hemosiderosis/pathology , Melanoma/pathology , Diagnosis, DifferentialABSTRACT
Objective To identify the expression of CD68-tumor-associated macrophages (TAMs) and proliferative marker Ki-67 in retroperitoneal malignant fibrous histiocytoma (MFH) and their clinical significance. Methods Clinical data about 35 patients with retroperitoneal MFH managed with surgery from February 2002 to December 2015 were retrospectively analyzed and all patients were followed up. There were 24 male and 11 female patients, and they were 18-71 years old, with mean age (53.0 ± 10.8) years old. Patients were divided into CD68 positive group (21 patients) and CD68 negative group (14 patients), while they were also divided into Ki-67 low expression (< 20%) group and Ki-67 high expression ( ≥ 20%) group, according to the immunohistochemical staining results. The overall survival time and all clinical data between two groups were compared. Kaplan-Meier estimations, Cox regression analysis, Fisher exact probabilities and Spearman correlations were performed. Results Of the 35 patients, 18 patients received radical resection, and 17 patients received palliative operation. The overall 1-, 3-, 5-year survival rates were 65.7%, 22.9%and 8.6%and the median survival was 17 (1-86) months. Factors associated with postoperative survival were FNCLCC grade (x2=7.002, P=0.008), modusoperandi of the tumor resection(x2=7.134, P=0.008), and CD68(x2=4.634, P=0.031) and Ki-67 overexpression (≥20%) (x2=8.898, P=0.003 ) . The difference between gender, age, tumor size, blood loss, removal of the joint organs and adjuvant therapy got no statistical significances (P > 0.05). Multivariate analysis showed that survival was associated with modusoperandi of the tumor resection and Ki-67 overexpression (P=0.003, 0.002, respectively). Conclusions Retroperitoneal malignant fibrous histiocytoma is a rare malignancy that display poor prognosis and high mortality. Complete resection remains the mainstream for retroperitoneal malignant fibrous histiocytoma. The patients' life span in CD68 positive or Ki-67 high expression is shorter. CD68 and Ki-67 plays a critical role in retroperitoneal malignant fibrous histiocytoma carcinogenesis and their high expression may be used as a potential survival predictor in patients with retroperitoneal MFH.