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.

Carbon ion radiotherapy for unresectable primary undifferentiated pleomorphic sarcoma of the 11th thoracic spine: a case report / Journal of Rural Medicine

Objective: Primary undifferentiated pleomorphic sarcoma (UPS) of the bone is rare. However, the common sites are the knee and proximal femur and humerus, while spinal involvement is rare. We report a case of primary UPS of the 11th thoracic vertebra, where corpectomy would have been difficult and extensive, treated with carbon ion radiotherapy.Case report: A 76-year-old man presented with an osteolytic tumor of the 11th thoracic vertebra on plain computed tomography (CT). The spinal cord was compressed and displaced posteriorly by the tumor on magnetic resonance imaging (MRI), and extraosseous extension was observed. An incisional biopsy was performed, and primary UPS of the 11th thoracic vertebra was diagnosed pathologically. Total en bloc spondylectomy was considered to be challenging because of the extraosseous extension and the patient’s age; thus, carbon ion radiotherapy (70.4 GyE / 32 fraction) was performed. Denosumab (120 mg) was administered subcutaneously every four weeks. No adjuvant chemotherapy was administered. Four years post-treatment, imaging revealed a compression fracture of the 11th thoracic vertebra, but there was no recurrence.Conclusion: Despite a poor prognosis and an aggressive course of UPS of the spine, the tumor continues to be controlled without local recurrence four years after carbon ion radiotherapy.

A Long-Term Survival Case after Successful Five-Fold Surgical Intervention for Recurrent Undifferentiated Pleomorphic Sarcoma in the Left Atrium / 日本心臓血管外科学会雑誌

Primary cardiac undifferentiated pleomorphic sarcoma is rare and is associated with very poor survival. We report a case of a 45-year-old female who presented with dyspnea on effort, in whom an echocardiographic exam showed a large mass in the left atrium and the tumor resection was performed. The pathological diagnosis of the resected tumor was undifferentiated pleomorphic sarcoma which subsequently recurred. The patient needed four re-surgeries, and chemotherapy with Pazopanib was performed. A long-term survival of 5 years after the initial surgery was achieved.

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.

Primary Chest Wall Sarcoma: Surgical Outcomes and Prognostic Factors / 대한흉부외과학회지

BACKGROUND: Primary chest wall sarcoma is a rare disease with limited reports of surgical resection. METHODS: This retrospective review included 41 patients with primary chest wall sarcoma who underwent chest wall resection and reconstruction from 2001 to 2015. The clinical, histologic, and surgical variables were collected and analyzed by univariate and multivariate Cox regression analyses for overall survival (OS) and recurrence-free survival (RFS). RESULTS: The OS rates at 5 and 10 years were 73% and 61%, respectively. The RFS rate at 10 years was 57.1%. Multivariate Cox regression analysis revealed old age (hazard ratio [HR], 5.16; 95% confidence interval [CI], 1.71–15.48) as a significant risk factor for death. A surgical resection margin distance of less than 1.5 cm (HR, 15.759; 95% CI, 1.78–139.46) and histologic grade III (HR, 28.36; 95% CI, 2.76–290.87) were independent risk factors for recurrence. CONCLUSION: Long-term OS and RFS after the surgical resection of primary chest wall sarcoma were clinically acceptable.

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.

Sinonasal Undifferentiated Pleomorphic Sarcoma in Five Patient Cases

Undifferentiated pleomorphic sarcoma (UPS) is a rare soft tissue sarcoma of the sinonasal area. Here, we present two primary cases of UPS and three post-irradiation sinonasal UPS cases. Imaging findings were misinterpreted by radiologists as representing other malignant tumors or recurrence of the primary tumor. Our cases indicate that post-irradiation UPS can originate within any part of the radiation field. Treatment outcomes of primary sinonasal UPS seem to be favorable if the tumor is treated aggressively, but the outcomes of post-irradiation sinonasal UPS may be poor if appropriate surgical margins cannot be obtained.

Prognostic correlation of MRI characteristics and Ki-67 expression in undifferentiated pleomorphic sarcoma / 实用放射学杂志

Objective To investigate the MRI features and Ki-67 expression and correlation with prognosis in undifferentiated pleomorphic sarcoma (UPS).Methods MRI and clinicopathological data of 34 cases with UPS proved by operation and pathology were analyzed retrospectively(with an average follow-up of 3 years).According to the presence of local recurrence,the data were divided into two groups:no recurrence group (15 cases)and recurrence group(19 cases).Two groups were analyzed according to age,tumor size,location,MRI features (lesions margin,hemorrhage,necrosis,pseudocapsule and perilesional characteristics)and expression of Ki-67.Results The results showed that two groups tumor’s size(P =0.002),location(P =0.025),pseudocapsule(P =0.025 ),peritumoral tissue inva-sion(P =0.008)and expression of Ki-67 (P =0.000)were of statistical correlation with tumor local recurrence.Conclusion Preoper-ative MRI can observe the characteristics of undifferentiated pleomorphic sarcoma,the relationship with the adjacent tissue and local invasive scope,which will be helpful for clinical diagnosis and decision of surgical plan.

Sarcoma Pleomórfico Indiferenciado de Maxilar de Alto Grado: Diagnóstico, Tratamiento y Pronóstico / Undifferentiated Pleomorphic Sarcoma of the Maxillary: Diagnosis, Treatment and Prognosis

Los sarcomas son neoplasias poco vistas en el territorio maxilofacial, donde la baja frecuencia de casos y los cambios de nomenclatura, han dificultado su estudio. Una de las variables de este tipo de lesiones corresponde a un tumor conocido como sarcoma pleomórfico indiferenciado, del cual se sabe muy poco pues se describe una incidencia de 1:1.000.000. Corresponde a un tumor maligno con características clínicas e imagenológicas de crecimiento invasivo y destructivo; la confirmación diagnóstica se realiza por medio de una biopsia en base al estudio histológico e inmunohistoquímico. Aparece principalmente en el tracto nasosinusal y su manejo terapéutico se basa en la cirugía con amplios márgenes de seguridad y radioterapia o quimioterapia según corresponda. No presenta metástasis locales pero si a distancia con gran afinidad por el pulmón. Se reporta un caso de sarcoma pleomórfico indiferenciado de alto grado de maxilar diagnosticado en la Unidad de Patología Oral y Maxilofacial del Hospital las Higueras de Talcahuano durante el año 2014, que a diferencia de la mayoría de los casos reportados, producto de su extensión, fue tratado con radioterapia paliativa. Además, se realizó una revisión de la literatura disponible en diversas bases de datos con lo cual se obtuvo información de casos ya reportados. Actualmente resulta difícil producto de la baja cantidad de casos descritos poder hacer proyecciones en cuanto a su pronóstico o evolución, sin embargo hay consenso de que el manejo interdisciplinario, diagnóstico precoz y la confirmación de este por medio de exámenes complementarios son fundamentales para su adecuado tratamiento.

Sarcomas are neoplastic lesions rarely observed in the maxillofacial region. Due to the few cases reported and to changes in nomenclature of these tumors their study has been difficult. One of the variants of this kind of lesion corresponds to a tumor currently known as undifferentiated pleomorphic sarcoma, which there is scarce knowledge of. In fact the incidence described is about 1:1.000.000. This one corresponds to a malignant tumor with image and clinical features as invasive and destructive growth, the confirmation of diagnosis is carried out by performing biopsy based on histologic and immunohistochemical studies. They appear mostly in the nose and sinusal tract. Its therapeutic management is based on surgery with wide security margins and radio or chemotherapy when warranted. No local metastases but distant ones are found and highly affined with lungs. We report a case of an advanced undifferentiated pleomorphic sarcoma diagnosed at the Oral and maxillofacial pathology unit of Hospital Las Higueras in Talcahuano at 2014, which in spite of most of the reported cases in literature; because of the extent it was treated with palliative radiotherapy. Also has been made review of the available literature in different databases with which information of the reported cases was obtained. Nowadays, due to the low number of cases described it is difficult to establish projections in terms of prognosis or evolution. However, there is consensus that interdisciplinary management, early diagnosis and confirmation with complementary exams are essential for an appropriate treatment.

Cutaneous Metastatic Undifferentiated Pleomorphic Sarcoma from a Mediastinal Sarcoma

Undifferentiated pleomorphic sarcoma, known as malignant fibrous histiocytoma, is a malignant neoplasm that arises in both soft tissue and bones. In 2002, the World Health Organization declassified malignant fibrous histocytoma as a formal diagnostic entity and renamed it 'undifferentiated pleomorphic sarcoma not otherwise specified.' It most commonly occurs in the lower extremities and rarely metastasizes cutaneously. We report a case of cutaneous metastatic undifferentiated pleomorphic sarcoma of the buttocks occurring in a 73-year-old man diagnosed with mediastinal sarcoma 4 years previously. He first noticed the mass approximately 2 months previously. Histological findings with immunomarkers led to a final diagnosis of cutaneous metastatic sarcoma from mediastinal undifferentiated pleomorphic sarcoma.

The Third Surgical Intervention for a Case of Recurrent Undifferentiated Pleomorphic Sarcoma of the Left Atrium / 日本心臓血管外科学会雑誌

We report a rare case of primary cardiac undifferentiated pleomorphic sarcoma with invasion to the posterior mediastinum, for which partial resection of the tumor in the left atrium had already been carried out twice. After remission for about three years, recurrence in the atrial wall involving the mitral valve posterior leaflet required a third surgical resection following mitral valve replacement.

Undifferentiated Pleomorphic Sarcoma in Mandible / 대한악안면성형재건외과학회지

Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, occurs commonly in the soft tissues in adult, but is rare in the maxillofacial region. It consists of undifferentiated mesenchymal tumor cells resembling histiocytes and fibroblasts. The purpose of this article is to report a case of UPS in the mandible. A 44-year-old patient presented with a painful growing mass in the mandible of two months' duration. Computed tomography and positron emission tomography-computed tomography revealed an ill-defined heterogenous, hypermetabolic mass about 4 cm in size in the left mandible invading adjacent soft tissues. A left mandiblulectomy and reconstruction with a fibular free flap were performed. Immunohistochemical study gave a diagnosis of UPS. The patient was referred for adjuvant chemotherapy after surgical removal of the tumor.

Left Atrial Undifferentiated Pleomorphic Sarcoma / 日本心臓血管外科学会雑誌

We report a case of a 24-year-old woman who presented with orthopnea, in whom an echocardiographic exam showed a very large mass in the left atrium. We diagnosed this as cardiac failure due to the tumor occupying it. Although the tumor malignancy remained unclear, we had to perform emergency surgery to excise the tumor. The tumor was excised in its entirety, including the interatrial septum and a large segment of the left atrial wall. We reconstructed them with the autologous pericardium. The pathological diagnosis was undifferentiated pleomorphic sarcoma. Conventional adjuvant chemotherapy and radiotherapy was performed. Primary cardiac malignant tumor prognosis is very poor, but she has survived over 1 year without recurrent symptoms after complete excision and adjuvant therapy. In addition to reporting this case, we discussed the diagnosis and treatment of undifferentiated pleomorphic sarcoma.

Primary Undifferentiated Pleomorphic Sarcoma of the Left Atrium that Presented as Acute Pulmonary Edema

A 37-year-old woman was admitted to Dong-A University Hospital for rapidly progressive congestive heart failure. Transthoracic echocardiography demonstrated a large mass with a stalk that appeared to be a myxoma on the posterior wall of the left atrium. However, the histological diagnosis was undifferentiated pleomorphic sarcoma. We report a case of primary undifferentiated pleomorphic sarcoma of the left atrium with acute pulmonary edema caused by mitral inflow obstruction.