Primary adrenal epithelioid sarcoma: a case report / 中华泌尿外科杂志

Adrenal epithelioid sarcoma is very rare in clinic. A case of epithelioid sarcoma of the right adrenal gland was reported in this paper. After physical examination, the patient was found to have a mass in the right adrenal area and underwent right adrenalectomy. The postoperative pathological diagnosis was right adrenal epithelioid sarcoma. Two months after adrenalectomy, positron emission tomography computed tomography(PET/CT) noted recurrence at the tumor bed and multiple metastases.The patient underwent chemotherapy combined with immunotherapy. After 16 months of follow-up, the disease was stable.

Sarcoma epitelioide de tipo proximal de vulva

El sarcoma epitelioide es un tipo raro de tumor mesenquimal. El sarcoma epitelioide de tipo proximal de vulva es un tumor maligno extremadamente raro de los tejidos blandos, con frecuentes recurrencias por su comportamiento agresivo. Debido a que sus características son similares a otras lesiones benignas, esto lleva a retrasos diagnósticos y terapéuticos. Las características histológicas de este tumor incluyen células epitelioides prominentes y aparición de rasgos rabdoides con marcadas atipias nucleares. Debido a su baja incidencia, no existen algoritmos diagnósticos basados en la evidencia ni recomendaciones publicadas para el tratamiento. El pronóstico es generalmente malo. Se presenta un caso de sarcoma epitelioide de tipo proximal de vulva.

Epithelioid sarcoma is a rare type of mesenchymal tumor. Proximal-type epithelioid sarcoma of the vulva is an extremely rare malignant tumor of the soft tissues with frequent recurrences due to its aggressive behaviour. Because its characteristics are similar to other benign lesions, this leads to diagnostic and therapeutic delays. Histologic features of this tumor include prominent epithelioid cells and the appearance of rhabdoid features with marked nuclear atypia. Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment. The prognosis is generally poor. A case of proximal type epithelioid sarcoma of the vulva is presented.

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.

Epithelioid sarcoma of paratesticular region: A case report of an unusual case

Primary tumors of the paratesticular region are rare, with sarcomas constituting a major proportion, particularly in the old age. The paratesticular region consists of mesothelial, various epithelial, and mesenchymal cells and may therefore give rise to a variety of tumors. Epithelioid sarcoma usually occurs at the distal extremities of young adults. The proximal-type variant is characterized by a more aggressive course and resistance to treatment. We herein report a case of 32 years male with scrotal swelling diagnosed as proximal-type epithelioid sarcoma of paratesticular region after histopathological examination and immunohistochemistry. To the best of our knowledge, this is only the second case of proximal epithelioid sarcoma of paratesticular region in the English literature. As these tumors are rare, it has been difficult to establish their optimal treatment. Also, these can be easily misdiagnosed as other epithelioid lesions.

Hemipelvectomy with laparoscopic abdominoperineal excision for epithelioid sarcoma treatment / Hemipelvectomia com amputação laparoscópica do reto para tratamento de sarcoma epitelioide

Abstract The epithelioid sarcoma (ES) is an unusual variant of the sarcoma, not reaching 1% of all soft tissue sarcomas. Initially reported in 1970, it has a more aggressive variant, which was classified as the "proximal-type" in 1997. These are aggressive tumors with high rates of distant metastasis and local relapses. Isolated radio and chemotherapy responses are poor and free-margin surgical resection is the treatment of choice.This is the case report of a 25-year-old male patient diagnosed with "proximal-type" ES in the perineal region. He underwent surgical resection in another institution and was later admitted to our institution with local recurrence with rectal and left thigh muscle invasion. Neoadjuvant radio-chemotherapy was performed, followed by laparoscopic abdominoperineal rectal resection and partial left hemipelvectomy associated with left lower-limb amputation. The patient had no postoperative complications and is currently undergoing outpatient follow-up. The anatomopathological analysis showed tumor-free margins.The "proximal-type" ES is a rare soft-tissue sarcoma subtype. High local recurrence, as well as the metastasis rates, makes its treatment a challenging task.

Resumo O sarcoma epitelióide (SE) é um subtipo incomum de sarcoma, não chegando a 1% dos sarcomas de partes moles. Foi inicialmente descrito em 1970, sendo que em 1997 uma variante mais agressiva foi classificada como "tipo proximal". São tumores agressivos, com altas taxas de metástases e recidiva local, com resposta pobre à quimioterapia e radioterapia isoladas. A ressecção cirúrgica com margens livres é o tratamento padrão.Apresentamos o caso de um paciente de 25 anos com SE tipo proximal avançado em região perineal, submetido a ressecção em outro serviço, evoluindo com recidiva local com invasão do canal anal e musculatura da coxa esquerda. Foi realizado tratamento radioquimioterápico neoadjuvante seguido de ressecção cirúrgica com amputação abdominoperineal do reto por videolaparoscopia associada a hemipelvectomia parcial esquerda incluindo o membro inferior esquerdo (MIE). O paciente apresentou boa evolução pós operatória e encontra-se em segmento ambulatorial. O anatomopatológico evidenciou margens cirúrgicas livres.O SE proximal é um tipo raro de sarcoma de partes moles, cujo tratamento curativo é desafiador, em virtude dos altos índices de recidiva local e metástases à distância.

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma - a rare vascular neoplasm with deceptive morphology and distinctive immunophenotype

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma is a rare, low grade vascular(endothelial) neoplasm typically presenting as multicentric, superficial to deep nodules in extremitieswith a slight tendency of affecting young adult males. We report a case of pseudomyogenichemangioendothelioma in a 15-year-old boy presenting initially with a 1 cm right thigh painlesscutaneous lump. The lump was excised with the clinical impression of a sebaceous cyst. On microscopy,a poorly circumscribed, mild to moderately atypical spindle cell lesion in fascicular and storiformpatterns with strikingly myoid-like eosinophilic cytoplasm was identified. The spindle cells werehighlighted by pancytokeratin AE1/AE3, CD31, and ERG with retained INI-1, while being negativefor MNF116, S100, CD34, EMA, desmin, SMA, caldesmon, myogenin, MyoD1, HHV-8 and CD163.Following the first diagnostic report, a positron emission tomography–computed tomography(PET-CT) scan revealed another 4 cm ill-defined nodule accompanied by a smaller adjacent 0.7cm ipsilateral satellite nodule within the right psoas muscle that displayed similar morphologyand immunophenotype as the cutaneous lump, supporting the multicentric feature of this uniqueentity. It is an uncommon yet increasingly recognised neoplasm of endothelial origin possessing amisleading myoid morphology and distinctive immunophenotype worth notifying.

Trial of Pazopanib in Proximal-type Epithelioid Sarcoma / 대한피부과학회지

No abstract available.

A rare case of multifocal pseudomyogenic hemangioendothelioma, involving soft tissues and bone, misdiagnosed as a rhabdomyosarcoma: Diagnostic and treatment implications.

Pseudomyogenic hemangioendothelioma (PHE) is an uncommon, but distinctive soft tissue tumor, characterized by multifocality. A 17‑year‑old male referred to us with progressively increasing multiple subcutaneous nodular lesions over his left leg and foot, reported elsewhere as a spindle cell rhabdomyosarcoma. On review, microscopy showed a cellular tumor comprising plump spindle cells arranged in loose fascicles with interspersed inflammatory cells. Tumor cells exhibited mild nuclear variation. Immunohistochemically, tumor cells expressed AE1/AE3, CD31, Fli‑1, and smooth muscle actin (SMA), confirming diagnosis of PHE. Whole‑body positron emission tomography–computed tomography (PET‑CT) scan revealed multiple, metabolically active, subcutaneous nodular lesions over the left lower leg and in the distal tibia. Subsequently, resection specimens from the various lesions and bone curettage also revealed features of PHE. Three months later, the patient developed multiple lesions over his fourth toe and left foot, for which he underwent tumor resections. At present, he is disease‑free. PHE is a locally aggressive soft tissue tumor characterized by multifocality, rarely bony involvement and can be misdiagnosed as a high‑grade sarcoma.

Gastrointestinal bleeding caused by epitheloid sarcoma: A case report

Epithelioid sarcoma (ES) of the small bowel is a rare gastrointestinal tumour. We report a case of gastrointestinal bleeding secondary to small bowel ES in a 55-year-old gentleman. After gastroscopy and colonoscopy failed to identify the source of bleeding, we proceeded with computed tomography angiogram of the mesentery, which revealed intraluminal blood clot in the distal jejunum with features of obstruction. This is a rare cause of obscure gastrointestinal bleeding and emphasises the need for additional evaluation in the presence of negative endoscopic findings.

Epithelioid Sarcoma in the Cervical Spine: A Case Report

Epithelioid sarcoma is a rare and highly malignant soft tissue neoplasm that most commonly occurs in the long bones. This uncommon tumor has a poor clinical outcome, and the modality of its treatment has not yet been fully established. The authors report an extremely rare presentation of epithelioid sarcoma in the cervical spine, along with its clinical progression, imaging, and pathology. The patient underwent three surgical procedures and adjuvant radiochemical management. He survived for 25 months with a good general condition and adapted well to his social activity. Systemic metastasis was not found, but the patient died of respiratory failure due to direct tracheal invasion of the tumor.

Report of 1 Case of Perineum Epithelioid Sarcoma / 浙江中医药大学学报

Objective Analyse and report the diagnosis and treatment process of vulvar epithelioid sarcoma, it provides a basis for rare case in clinic. [Methods] Through the analysis of the patient's il ness, treatment, and pathology examination, and the effect of the treatment of prognosis of vulvar epithelioid sarcoma of the summary, truly record the occurrence of vulvar epithelioid sarcoma development, treatment and prognosis. [Results] The intraoperative pathologic examination in patients diagnosed the vulvar epithelioid sarcoma, and it used the removal of expanding. Now the patient is not recurred. [Conclusion] Vulvar epithelioid sarcoma is clinical y rare, needs combined with immunohistochemical examination at the time of diagnosis, removal of local expansion is the effective method to treat, it is not exactly curative effect of chemotherapy and radiotherapy.

A case of epithelioid sarcoma arising in the vulva / 부인종양

Epithelioid sarcoma of vulva is extremely rare. Only 20 cases have been reported in the literature so far. Early diagnosis is difficult because of its benign appearance as a painless subcutaneous nodule. Therefore treatment can be delayed, although epithelioid sarcoma requires prompt surgery. We report a case of epithelioid sarcoma that occurred in the left vulva with a brief review of literature.

Epithelioid Sarcoma in the Foot: A Case Report / 대한족부족관절학회지

Epithelioid sarcoma is a rare tumor which is usually presented with a nontender nodule on a distal extremity. It is sometimes confused with granulomatous process or chronic inflammation. We report of a case of epithelioid sarcoma on a foot of an adult male, which progressed rapidly.

Imprint Cytologic Features of Epithelioid Sarcoma: A Case Report / 대한세포병리학회지

Epithelioid sarcoma is a malignant soft tissue neoplasm with an uncertain histogenesis. We report the imprint cytologic features of epithelioid sarcoma in the left shoulder of a 29-year-old male patient. Imprint cytologic findings showed dissociated and loose aggregates of anaplastic epithelioid cells on the necrotic, bloody, and inflammatory background. Tumor cells were round to polygonal shaped. Tumor cells had vesicular nuclei with abundant cytoplasm. The nuclei were irregular in shape and often eccentrically located. Some tumor cells were oval to spindle shaped. Binucleated and multinucleated cells were found. Intracytoplasmic vacuoles were present. On immunohistochemical stain, the tumor cells were positive for epithelial membrane antigen, vimentin, and CD34.

A Case of Epithelioid Sarcoma / 대한피부과학회지

Epithelioid sarcoma is a rare soft tissue neoplasm of uncertain origin and occurs most commonly on the distal extremities of young adult males. It usually presents as a slowly growing dermal or subcutaneous nodule, however it is characterized by multiple recurrences and metastases. Its slowly growing pattern and confusing histopathologic findings often result in inaccurate or delayed diagnosis. We describe a 38-year-old female of epithelioid sarcoma on the hand, emphasizing that clinicians should consider the possibility of this disease in case of nodular or ulcerated lesion of the extremities, especially distal upper extremities.

A Case of Epithelioid Sarcoma in a Child

Epithelioid sarcoma is a rare distinctive malignant neoplasm of soft tissue with frequent recurrences and late metastases. It typically affects young adult males, and epithelioid sarcoma arising in the first decade of life is very rare. Epithelioid sarcoma may be confused with a wide range of benign, especially granulomatous, diseases and other malignancies, but the differentiation can be made, in general, by the characteristic clinical, histopathologic, and immunohistochemical features. We report herein a case of epithelioid sarcoma arising on the volar side of the right 5th finger in an 8-year-old boy. To our knowledge, this is the first report of epithelioid sarcoma in a Korean child.

Aspiration Cytology of Epithelioid Sarcoma: A Case Report / 대한세포병리학회지

Cytologic features of the fine needle aspiration of metastatic epithelioid sarcoma are presente. The patient was a 48 year-old male who had been sufferring from recurrent and metastatic tumors of the right upper extremity for 6 years. The aspirate from the axillary mass cytologically consisted of isolated large polygonal cells with abundant, cyanophilic, slightly granular cytoplasm. Eccentrically located large round nuclei showed finely dispersed chromatin, small nucleoli, occasional convolution and folding of nuclear membrane, and frequent binucleation. Although both histologic and cytologic similarity of epithelioid sarcoma to squamous cell carcinoma is well known, findings of cyanophilic granular cytoplasm, convoluted nuclei, and binucleation appeared to be the differential points.

A Case of Metastatic Epithelioid Sarcoma

Epithelioid sarcoma(ES) is a very rare, unique soft tissue sarcoma, which was first fully described by Enzinger in 1970. The tumor chiefly occurs in young adults and affects the soft tissue of the distal extremity. A 46-year-old male patient had slowly growing masses on his left plantar surface, which were diagnosed as ES and mass excision and radiation therapy were done. Eight months later, about 1 × 1 cm sized, well defined multiple erythematous nodules on his scalp were discovered. Although he was treated with chemotherapy, no improvement was gained.

Two Cases of Epithelioid Sarcoma with Immunohistochemical Study

Two typical cases of epithelioid sarcoma were examined by immunohistochemical stain using antibodies to epithelial membrane antigen, carcinoembryonic antigen, vimentin and cytokeratin. Both cases showed positive reactivity for the four kinds of antibodies. These results point to the fact that epithelioid sarcoma simultaneously expresses epithelial markers and characteristic mesenchymal phenotypes. Epithelioid sarcoma appears to be a tumor derived from a multipotential mesenchymal cell with multidirectional differentiation.

Clinicopathologic study of epithelioid sarcoma / 重庆医科大学学报

Objective:To study the clinicopathologic features、diagnosis and differential diagnosis of epithelioid sarcoma.Methods:Two cases of epithelioid sarcoma were collected according to the classification of soft tissue and bone tumors(WHO,2002) and were studied by hematoxylin-eosin and immunohistochemical staining.Results:One case was proximal type epithelioid sarcoma,and the other distal type epithelioid sarcoma.The case of distal type epithelioid sarcoma was female,twenty eight years old,and the site was the left leg;histological structure was the typical pseudo-granuloma formation,the tumor cells resembled epithelioid cells,with necrosis in the center.The case of proximal type epithelioid sarcoma was female,thirty six years old,and the site was the cervix;nodal formation can be observed,the tumor cells resembled spindle cells,abd the extent of necrosis was bigger.For the immunohistochemical staining,expressions of Vimentin、EMA and CK antigen were observed in the 2 cases,while expressions of Desmin、NSE and S-100 antigen were negative.Moreover,expressions of low CK and CD34 antigen were also observed in the case of distal type epithelioid sarcoma.On the other hand,expressions of Actin、SMA and HMB-45 antigen were not observed in the case of proximal type epithelioid sarcoma.Conclusions:Epithelioid sarcoma is a rare maliglant tumor of soft tissue,It's the key for diagnosis and differential diagnosis to combine clinic、histological structure、immunophenotype and prognosis together.