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.

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

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

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.

Rapidly progressive malignant fibrous histiocytoma of right atrium: a rare case report

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.

Primary Undifferentiated Pleomorphic Sarcoma of the Colon Mesentery

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.

Long-Term Survival after Wide Resection of Malignant Fibrous Histiocytoma of the Chest Wall / 대한흉부외과학회지

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.

A Case of Myxofibrosarcoma in the Cheek / 대한이비인후과학회지

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.

Histiocitoma fibroso maligno metastásico en estómago / Metastatic malignant fibrous histiocytoma in the stomach

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)

The expression and clinical significance of CD68 and Ki-67 in retroperitoneal malignant fibrous histiocytoma / 中国医师进修杂志

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.

The expression and clinical significance of CD68 and Ki-67 in retroperitoneal malignant fibrous histiocytoma / 中国医师进修杂志

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.

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)

Angiomatoid fibrous histiocytoma: Clinicopathological spectrum of five cases, including EWSR1‑CREB1 positive result in a single case.

Background: Angiomatoid fibrous histiocytoma (AFH) is an unusual soft tissue tumor (STT), characterized by recurrences, but rarely metastasis. Later, certain molecular signatures have been identified underlying this tumor, which at times, is either underdiagnosed as a benign vascular tumor, or over diagnosed as a high‑grade pleomorphic sarcoma, including a malignant fibrous histiocytoma. Materials and Methods: Over a 14‑year‑period, five diagnosed cases of AFH were analyzed. Results: Five tumors occurred in three males and two females, over a wide age‑range (median = 21, mean = 30 years); mostly in the extremities (4) (80%). Microscopically, most tumors were circumscribed, comprising large, blood‑filed spaces with surrounding histiocytic cells and a “cuff” of lymphoplasmacytic cells. Three tumors revealed solid growth pattern with polygonal to spindle cells, including myxoid matrix in one of these tumors. On molecular analysis, this tumor exhibited EWS‑CREB transcript. Immunohistochemically, various tumors were positive for CD68 (n = 2/2), epithelial membrane antigen (n = 3/4), CD99/MIC2 (n = 2/3), and desmin (n = 1/4). All tumors were surgically excised. On follow‑up (n = 2), a single patient, who underwent wide‑excision was free‑of‑disease (24 months), while another patient had a recurrence 4 months post tumor excision. Conclusions: This forms as the first documented series on clinicopathological features of AFH, a rare STT, from our country. Significant clinicopathological features include younger age, extremities as commonest site and histopathological appearance of blood‑filled spaces with surrounding “cuff” of histiocytic cells and lymphocytes. Tumors with unusual histopathological tumor patterns require molecular confirmation. Surgical resection remains the treatment mainstay.

Case of Pleomorphic Dermal Sarcoma of the Eyelid Treated with Micrographic Surgery and Secondary Intention Healing

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face.

Case of Pleomorphic Dermal Sarcoma of the Eyelid Treated with Micrographic Surgery and Secondary Intention Healing

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face.

Fibrohistiocitoma maligno mamario: descripción de un caso

El fibrohistiocitoma maligno de mama es una entidad muy poco frecuente que debuta como un nódulo mamario de reciente aparición y de crecimiento relativamente rápido. El diagnóstico debe establecerse mediante una muestra histológica, debido a que las pruebas de imagen suelen ser inespecíficas. El tratamiento habitual es la mastectomía con o sin vaciamiento axilar y la terapia adyuvante (quimioterapia y radioterapia) suele tener poca efectividad en esta patología.

Malignant fibrous histiocytoma of the breast is arare entity that debuts as a breast lump of recent onset and relatively fast growth. Diagnosis is by histology because imaging tests are often nonspecific. Usual treatment is mastectomywith orwithout axillary dissection; acjuvant therapy (chemotherapy and radiotherapy) often have limited effectiveness in this pathology.

Neoplasmas em suínos: 37 casos / Neoplasia in swine: 37 cases

Neoplasmas em suínos são raros. Esse trabalho descreve os neoplasmas encontrados em suínos na rotina diagnóstica de um laboratório de patologia veterinária localizado na Região Central do Rio Grande do Sul. Durante um período de 49 anos, 2.266 casos de várias afecções em suínos foram encontrados, dos quais 37 (1,6%) eram neoplasmas. Em ordem decrescente de frequência, os seguintes neoplasmas foram encontrados: Linfoma (11/37), nefroblastoma (11/37), melanoma (8/37) e papiloma (2/37). Adicionalmente, um caso de cada um dos seguintes tumores foi observado: Adenoma hepatocelular, carcinoma hepatocelular, colangiocarcinoma, histiocitoma fibroso maligno e sarcoma granulocítico. O aspecto macroscópico e histológico desses tumores é descrito e a sua epidemiologia é comparada com os dados disponíveis na literatura para neoplasia suína.

Neoplasms in swine are rare. This paper describes neoplasms found in swine in the diagnostic routine of a veterinary pathology laboratory in the Central Region of Rio Grande do Sul, Brazil during a 49-year period, during which 2,266 cases of the various affections in swine were diagnosed. Of those 37 cases (1.6%) were neoplasms. In decreasing order of prevalence, the following neoplasms were found: lymphoma (11 out of 37 cases), nephroblastoma (11/37), melanoma (8/37), and papilloma (2/37). Adenoma hepatocelular, carcinoma hepatocelular, cholangiocarcinoma, malignant fibrous histiocytoma, granulocytic sarcoma were each found in one case out of the 37 cases. The gross and histological aspects of these tumors are described and their epidemiology is compared with the data available in the literature for neoplasia in swine.

CT findings of primary malignant fibrous histiocytoma of the liver / 中华肝胆外科杂志

Objective To investigate the CT imaging characteristics of primary malignant fibrous histiocytoma (MFH) of the liver.Methods The CT, clinical and histopathologic data of 6 patients with MFH were analyzed retrospectively.All the cases were confirmed by surgery and histopathological examinations.Results The tumors were located in the right liver in 4 patients and the left liver in 2 patients.The maximum diameter of the tumors ranged from 5 to 20 cm.All the tumors were unevenly hypodense on plain CT scan.Four tumors had relatively well-defined margins, while 2 tumors had ill-defined margins.After contrast enhancement, the peripheral and internal divisions of the three storiform-pleomorptic MFH showed progressive enhancement.The peripheral and internal divisions of the two inflammatory MFH were obviously enhanced in the arterial phase, and the enhancement decreased in the portal and delayed phases.The hypodense areas of the one myxiod MFH were not enhanced, but the internal floc and divisions were progressive enhanced.On gross pathology, the tumors were round or irregular in shape, most had a complete capsule with multiple surrounding vessels.These findings correlated well with the CT findings.Conclusions CT findings of primary Hepatic MFH have some characteristics.The enhancement pattern is manifested in a fast wash-in and slow wash-out pattern.However, the final diagnosis still relies on histopathological examination.

Intracranial Undifferentiated Sarcoma Arising from a Low-Grade Glioma: A Case Report and Literature Review

Undifferentiated sarcomas are rarely identified in the intracranial region. A 23-year-old man was admitted with a chief complaint of headache. Initial magnetic resonance images showed signs of low-grade glioma in the frontal lobe. Stereotactic biopsy was performed, and a diagnosis of diffuse astrocytoma was confirmed. Three months later, the patient presented with a high-grade tumor as seen on imaging studies. He underwent total resection of the tumor and histopathological tests identified an undifferentiated sarcoma. The patient died eight months later due to massive tumor bleeding. To the best of our knowledge, this is the first report of undifferentiated sarcoma arising from low-grade glioma without any chemotherapy or radiotherapy.