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Introduction and importance Undifferentiated pleomorphic sarcoma (UPS) is one of the most common sarcomas affecting elderly patients, majority of UPS usually in the extremities, trunk, and retroperitoneum. However, its rarely observed in the digestive system. There are minimal data published regarding this topic. Case presentation Here we present a 58 years old female patient with a rare case of the pleomorphic sarcoma of retro-gastric mass measures about (24.7 × 23.5 × 17.4 cm) who underwent exploration laparotomy founded a freely and mobile retro-gastric mass measuring 30 × 20 cm with a cystic and solid component. The mass was freely and mobile located with no true gastric relation is the first one to be reported. Clinical discussion The undifferentiated pleomorphic sarcomas or MFH has been regarded as the most common soft tissue sarcoma for many years (Kabashima et al., 2017). The incidence increases with age, with an inclusive incidence of about 1–2 cases per 100,000 patients per year, predominately 1.2:1 for males. Undifferentiated pleomorphic sarcoma of the stomach is rare and counted to have a worse prognosis when compared to the other types of pleomorphic malignant fibrous histiocytoma. Conclusion We present a rare case of high-grade undifferentiated pleomorphic sarcoma of the retro-gastric mass with no invasion to the surrounding structures, managed with surgical resection of the tumor., Highlights • A rare case of the of retro-gastric sarcoma with no true direct relation to the stomach, the first case reported • Undifferentiated pleomorphic sarcoma is usually reported in the extremities, trunk, and retroperitoneum and observed in the digestive system. • The potential of adjuvant radiotherapy for USP showed no sufficient data was supporting such treatment

Gastrointestinal tract involvement in undifferentiated pleomorphic sarcoma (UPS) is extremely rare. To the authors’ knowledge, only 21 cases of primary small bowel UPS have been reported in the literature available in English. Reported CT findings in primary small bowel UPS have been nonspecific, and MRI findings have been reported in only one case. The present article describes a case involving a 72-year-old male with histologically confirmed primary UPS arising from the ileum, focusing on both CT and magnetic resonance enterography findings. On CT, primary small bowel UPS was noted as a heterogeneously enhanced small bowel mass without obstruction. Magnetic resonance enterography revealed heterogeneous intermediate T1 and T2 signal intensity, with hemorrhagic or necrotic foci within the mass and heterogeneous enhancement. The differential diagnosis included malignant gastrointestinal tumor; however, the prognosis of UPS is worse, with higher incidences of extra-abdominal metastasis.

Background: Pleomorphic Malignant Fibrous histiocytoma (MFH) is a rare neoplasms of the soft tissue and bone composed of fibroblastic and histiocytic components with mitosis figures, nuclear pleomorphism and anaplasia. Case Presentation: We presented a rare case of MFH in 44 year old woman with history of systemic erythematosus lupus and seizures. Treated with prednisone. The cerebral TAC showed a temporal mass. Craniotomy was performed and the examination of the biopsy sample revealed a giant, pleomorphic and atypical cells. Immunohistochemical analysis showed positivity for vimentin, CD68, Fascin, lysosyme and MIB-1 labeling index of 20%. Pleomorphic malignant fibrous histiocytoma was diagnosed, with involvement of the overlying temporal lobe. We report a case of MFH of the left temporal in adult. Discussion: The neuroimaging and intra-operative opinion were of a meningioma. Histopathology, in conjunction with immunohistochemical evaluation and proliferation marker, confirmed the diagnosis. This is the firth case reported of PMFH associated to systemic lupus erythematous and different histological patterns.

We present a case of pleomorphic malignant fibrous histiocytoma arising from the left forearm in a 45-year-old man who had undergone resection and radiotherapy for a tumor 3 years previously. At his first visit, he had multiple lung and bone metastases. Although these metastases responded well to systemic chemotherapy, brain metastases newly appeared and caused the death of the patient. These findings demonstrate that individual sarcomatous metastatic organs exhibit different sensitivities to chemotherapy. The mechanism of this phenomenon is discussed with a review of previous reports. It is suggested that the blood-brain barrier may play an important role in sensitivity to chemotherapy.

Atypical fibroxanthoma (AFX), a benign lesion, and pleomorphic malignant fibrous histiocytoma (MFH) are thought to represent points along the same neoplastic spectrum but with different prognoses and treatments. Diagnosis based on histology and clinical parameters alone is sometimes difficult, and a reliable cost-effective immunohistochemical marker to help distinguish these lesions would be beneficial. The diagnosis of AFX or MFH was based upon published clinical and microscopic criteria. Formalin-fixed, paraffin-embedded tissues of 17 cases of AFX and 26 cases of MFH were immunostained with monoclonal antibody to CD99. For all cases, CD99 expression was scored on a four-tiered scale: negative, weak (1+), moderate (2+), or strong (3+). Two pathologists blinded to tumor diagnoses and type of immunostain evaluated each case independently. The interobserver correlation coefficient was calculated. Seventeen patients with AFX (16 males and one female; mean age = 79) and 26 patients with MFH (16 males and 10 females; mean age = 60) were included. AFX lesions were from the head and the face, mean size = 1.5 cm, and MFH lesions were from the head, the neck, the trunk, and the upper/lower extremities, mean size = 5.2 cm. The 17 cases of AFX demonstrated moderate or strong (2 to 3+) immunoreactivity with CD99, compared to nine of 26 (35%) MFH cases (chi-square = 18.38; p < 0.001; interobserver correlation coefficient = 0.83). Of these, 16 of 17 (94%) AFX cases stained diffusely with CD99, while only four of 26 (15%) MFH cases stained diffusely. Control slides were adequate. Our study demonstrated that CD99 can help distinguish AFX from MFH, in addition to other immunohistochemistry as well as clinical and histologic criteria.

Diaphyseal medullary stenosis (DMS) is an extremely rare hereditary bone dysplasia, which was first described by Arnold in 1973. DMS has a high incidence of pleomorphic malignant fibrous histiocytoma (MFH). In this paper, we report the imaging findings of DMS with pleomorphic MFH of the bone, mainly describing 99mTc hydroxymethylenediphosphonate (HMDP) and thallium-201 (201Tl) chloride scintigraphy findings. On 99mTc HMDP scintigraphy, focal increased uptake area of the right femur corresponded to the area of bone marrow invasion of the tumor and bone infarction. The mechanism of the uptake of 99mTc HMDP to the extraosseous lesion was not clear. On 201Tl chloride scintigraphy, the increased uptake of the periphery of the mass seemed to reflect the aggressiveness of invasion and the cellularity.

Integrated 2-deoxy-2-F18-fluoro-D-glucose positron emission tomography combined with computed tomography (FDG-PET/CT) has been used in the field of soft tissue sarcoma. We report an 81-year-old man with dedifferentiated liposarcoma in the left thigh, which was composed of well-differentiated liposarcoma and pleomorphic malignant fibrous histiocytoma. As well as other radiological modalities, FDG-PET was able to demonstrate a biphasic signal pattern composed of well-differentiated liposarcoma and dedifferentiated area, being consistent with the histological grade of malignancy.

We report a case of dedifferentiated leiomyosarcoma. The patient was a 56 year-old man. The tumor was local recurrence in the right greater pectoral muscle after radiotherapy and chemotherapy. Tumor showed typical moderately leiomyosarcoma with dedifferentiated component. This component was revealed as pleomorphic malignant fibrous histiocytoma as a result of immunohistochemistry. These results indicated dedifferentiated components derive from loose muscle features due to dedifferentiation. We diagnosed this case as dedifferentiated leiomyosarcoma.

Primary sarcoma of the penis has an extremely low incidence, and its diagnosis and treatment are unclear. A 55-year-old man presented with an oval-shaped mass of the glans penis, which was treated by a wide excision. The pathologic result revealed an undifferentiated high-grade sarcoma, which was diagnosed as a pleomorphic malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma based on the World Health Organization classification. There was no recurrence at the 12-months postoperative follow-up.

Atypical fibroxanthoma (AFX) is a superficial variant of pleomorphic malignant fibrous histiocytoma. Clear cell atypical fibroxanthoma (CCAFXA) is a rare variant of atypical fibroxanthoma. A 74-year-old male patient presented with a rapidly growing nodule on the shoulder, which had appeared over a 5-months period. Lesion was excised by the plastic surgeon. Microscopic examination showed an ulcerated nodule composed of pleomorphic spindled and polygonal cells with clear or vacuolated cytoplasm. The neoplastic cells were stained positively with CD68 and CD10 and were stained negative with S-100, Melan A, muscle-specific actin, or pan-cytokeratin. Final diagnosis was clear cell atypical fibroxanthoma. CCAFXA should be differentiated from other clear-cell neoplasms of the skin. Best of our knowledge only 11 cases CCAFXA of have been reported in published studies till date. Herein, we reported 12th case in literature of CCAFXA and review of the literature.

The authors report the clinico-pathological features of a rare case of pleomorphic malignant fibrous histiocytoma of the spermatic cord. A review on the topic is provided.

Primary pleomorphic malignant fibrous histiocytoma of the heart is rare. The present study was performed to study the clinical and pathological features of the disease. We describe two rare cases of primary cardiac malignant fibrous histiocytoma and review the published individual data of the patients. Both patients complained of dyspnea, and underwent palliative tumor resection. However, they died several months after surgery. A thorough literature review with clinical presentations, diagnostic features, treatment, and outcomes was done. We have for the first time analyzed the factors related to the survival of malignant fibrous histiocytoma. It is usually difficult to make an appropriate preoperative diagnosis. Despite complete surgical resection and aggressive chemotherapy and radiotherapy, the prognosis is still poor.

We report a case of an 83-year-old previously healthy female patient presenting with a swiftly evolving erythematous violaceous, infiltrative, ulcerated onion like mass with hyperkeratotic surface on the left ear lobe. The lesion was excised and resulted as an atypical fibroxanthoma, an extremely rare neoplastic growth, being a superficial variant of pleomorphic malignant fibrous histiocytoma. A brief review of dia-gnosis, treatment and prognosis is discussed.

Pleomorphic malignant fibrous histiocytoma (MFH), also known as undifferentiated high-grade pleomorphic sarcoma according to the latest World Health Organization classification, is a diagnosis of exclusion and extremely rare in adult scrotal/paratesticular region. Clinical presentation of scrotal/paratesticular pleomorphic MFH is usually a painless and gradual scrotal swelling. We report a case of scrotal MFH in a 63-year-old man who presented as Fournier gangrene after 10-month painful scrotal swelling and multiple procedures. The specimen of emergent debridement was submitted for pathologic and bacteriologic examination. Microscopically, the lesion had marked architectural and cytologic pleomorphism. The neoplastic cells were positive for vimentin, but negative for all lineage-specific markers. Fluorescence in-situ hybridization showed an aneuploid karyotype and negative results for lipomatous tumor abnormalities. Bacterial cultures of the specimen showed extensive growth of virulent polymicrobes. The diagnosis of scrotal/paratesticular pleomorphic MFH with concurrent Fournier gangrene was made. Thoracic computed tomography scan showed bilateral multiple pulmonary nodules. The patient died 1 month later.

Pleomorphic malignant fibrous histiocytoma (MFH) is regarded as the most common soft tissue sarcoma of adulthood, but no definable criteria exist for its diagnosis. Possibly its only distinctive feature is its apparent lack of specific differentiation. To determine the validity of pleomorphic MFH, 159 tumors diagnosed as pleomorphic sarcomas have been reassessed morphologically, immunohistochemically, and ultrastructurally, where possible. Of these 97 cases (63%) proved to be specific sarcomas other than MFH, 20 proved to be nonmesenchymal neoplasms, and 42 were unclassifiable (of which 21 were either small biopsies or subtotally necrotic). Only 13% of these cases were eligible for consideration as MFH, but these showed no reproducible histological differences from the other tumors studied, nor was this group morphologically consistent. These tumors showed no evidence of true monocyte/macrophage differentiation. It is postulated that pleomorphic MFH is a noncohesive heterogeneous group of poorly differentiated neoplasms, a term that has become a meaningless diagnosis of convenience. With sufficient effort, a specific line of differentiation can be identified in the majority of pleomorphic malignant soft tissue tumors; with advances in investigative technology, the proportion that remain unclassifiable is very likely to diminish further in the future.

10556 Background: Myxofibrosarcoma and pleomorphic malignant fibrous histiocytoma (PMFH) are aggressive genetically complex sarcomas. We investigated the association of percent myxoid component (MC...

Explanations for the disparate behavior of atypical fibroxanthoma (AFX) as compared with pleomorphic malignant fibrous histiocytoma (MFH) have included the proposition that the former is a pseudosarcoma. Nonetheless, these tumors are now widely regarded as the same process, but with AFX behaving benignly by virtue of its superficial location. However, a neoplasm's metastastatic potential has been proposed to be related to apoptosis. Therefore, the aim of the present study was to examine apoptotic counts, in conjunction with two important regulators of apoptosis: p53 and bcl-2, to determine if a distinction exists that may account for the different outcomes of these lesions. There was no significant statistical difference between eight AFX and nine pleomorphic MFH in terms of apoptotic behavior, proliferative indexes, p53 protein expression, or presence of bcl-2 product. Therefore, our results further support the contention that AFX should be regarded as a form of pleomorphic MFH, which demonstrates low malignant potential by virtue of its location in readily accessible sites.

The nature and nosologic status of malignant fibrous histiocytoma (MFH), or at least its most common pleomorphic variant, are a matter of controversy and it is possible that this entity represents the shared morphologic phenotype of a range of other dedifferentiated neoplasms. An unusual case of malignant peripheral nerve sheath tumour which very closely mimicked pleomorphic MFH is presented. Its true nature was only disclosed by ultrastructural examination. A further interesting and previously unreported feature in tumours of this type was the presence of annulate lamellae in many of the tumour cells. This case adds weight to the assertion that the light microscopic features of pleomorphic MFH may not be specific.

Eight cases of benign cutaneous fibrous histiocytoma containing scattered, bizarre, pleomorphic cells of multinucleate or histiocyte-like type are described. All arose in adults and four had originally been diagnosed as sarcomas. Lesions of this type are not well-known and merit wider recognition in order to avoid inappropriate treatment. Differential diagnosis principally includes atypical fibroxanthoma and pleomorphic malignant fibrous histiocytoma.

It is a small nodular lesion of the derma, which is histologically nearly the same as a pleomorphic malignant fibrous histiocytoma. It is differentiated by its superficiality (it may just, but not extensively, extend into the subcutaneous tissue), by its absence of necrosis and, in particular, by its low aggressiveness.

A correlative cytologic and histologic study of 40 cases of histologically highly pleomorphic malignant fibrous histiocytoma (MFH) is presented. The fine-needle aspiration biopsy was performed preoperatively, and a diagnosis of malignant soft-tissue tumor could be established in all cases. The cytologic and histologic features corresponded well with each other. The two main cell types were mono- and multinucleated, large polymorphic, often bizarre, histiocyte-like cells and atypical fibroblast-like cells. For a correct diagnosis of pleomorphic MFH, it is important to recognize atypical large polymorphic tumor cells showing signs of phagocytosis: prominent cytoplasmic vacuolization, cell debris or even well-preserved cells within the tumor cell cytoplasm. Phagocytic activity was easily demonstrated in air-dried and May-Grunwald Giemsa-stained material. The differential diagnosis of MFH as opposed to other soft-tissue sarcomas and pleomorphic carcinomas is discussed. Diagn Cytopathol 1986;2:46-54.

An exceptional case of pleomorphic malignant fibrous histiocytoma of the larynx occurring in a 68-year-old male patient is reported. A significant histological documentation is given. Problems connected with histogenesis, treatment and prognosis are dealt with. Stress is laid on the need to establish differential diagnosis from other neoplasms, in particular liposarcoma and spindle-cell carcinoma.