Epithelioid sarcoma is a rare soft tissue neoplasm of uncertain lineage that usually arises in the distal extremities of adults, presents a high rate of recurrences and metastases and frequently poses diagnostic dilemmas. [ncbi.nlm.nih.gov]

CASE SUMMARY A 13-year-old Hispanic girl without significant medical history presented with 6 months of left-hand swelling and pain. [appliedradiology.com]

Four months after initial presentation the nodule measured 1.4×1 cm (B). Case Report A 23-year-old man presented with a nonhealing lesion on the right palm. [mdedge.com]

Case Presentation A 67 year old man presented with a 3.2 cm intraparenchymal left upper lobe lung coin-lesion identified incidentally on a computerized tomography (CT) study (Figures 1A,B). [austinpublishinggroup.com]

The present case highlights the difficulties in diagnosing this rare entity because of its uncommon location and histological mimics which can be overcome by judicious use of IHC markers. [ijpmonline.org]

• Soft Tissue Mass

  CONCLUSION Soft-tissue masses often present with a nonpecific imaging appearance, and tend to have a wide differential diagnosis. [appliedradiology.com]

  They account for 2.5-10% of all soft tissue sarcoma 2-4. The presentation is most often with a slowly-enlarging soft tissue mass which may have been noted for some years and gives a false impression of a benign process 4. [radiopaedia.org]

  Keys To The Initial Evaluation Of A Patient With A Soft Tissue Mass The initial evaluation should include a thorough history. One should consider all soft tissue masses of the extremities to be a primary sarcoma until proven to the contrary. [podiatrytoday.com]

  Roper and Rebecca Stein-Wexler, Soft Tissue Masses, Pediatric Orthopedic Imaging, 10.1007/978-3-642-45381-6_22, (715-769), (2014). Andrew M. Davidoff, Israel Fernandez-Pineda, Victor M. Santana and Stephen J. [doi.org]

  MDCT revealed multiple bulky, lobulated, intraperitoneal soft tissue masses of varying sizes ranging from 4 × 3 × 3 cm to 15 × 8.5 × 6.5 cm. [omicsonline.org]

• Pleural Effusion

  None of the patients presented with pleural effusion. Our patient represents the first reported case of spontaneous pneumothorax and pleural effusion in both lungs in ES. [kjim.org]

  Thorascopic surgery was performed for a left pleural effusion and metastases to the left parietal pleura and adjacent soft tissue were identified. The patient was subsequently lost to follow-up. [mdedge.com]

  The actuarial 5-year distant metastasis rate was 40%, with pleuropulmonary metastases the most common site of metastatic disease, and 35% of pleuropulmonary metastases presented with pleural effusion. [thedoctorsdoctor.com]

  Ali, Metastatic proximal epithelioid sarcoma in pleural effusion: Cytopathologic findings and differential diagnosis, Diagnostic Cytopathology, 41, 6, (542-545), (2011). Ranjith Babu, Isaac O. Karikari, Thomas J. Cummings, Oren N. [doi.org]

• Ulcer

  Clinically, like our case, epithelioid sarcoma may present as a relatively small, ulcerated, single, cutaneous nodule. [oapublishinglondon.com]

  There was an erythematous nodule on the right palm measuring 2.0 x 1.0 cm, with ulceration and scarring [Figure - 1]. The ulcer was irregular with punched out and raised edges. The base was firm and the floor was covered with granulation tissue. [ijdvl.com]

  Fig 1: Erythematous ill defined multi-nodular firm mass with areas of ulceration affecting the left thumb and the thenar eminence. [edoj.org.eg]

  A skin biopsy of the ulcerated lesion revealed proliferation of pleomorphic epithelioid cells with eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli. [scielo.br]

  Cyril Fisher, MD, DSc, FRCPath Clinical photograph shows several rounded lesions with raised red margins and central “targetoid” ulceration on the forearm of a 23-year-old man. Nonhealing ulcers are typical of epithelioid sarcoma. [basicmedicalkey.com]

• Subcutaneous Mass

  mass Classic ES Dermal or subcutaneous nodule Proximal ES Subcutaneous or deeper mass Can appear more rapidly Ulcer Nonhealing ulcer with raised margin Natural History Classic ES Persistent and multiple recurrences Successive lesions often recur and [basicmedicalkey.com]

  The tumors presented as deep soft-tissue or subcutaneous masses on the inguinal region in five, the thigh in four, the vulva in three, the axilla in three, and one each in the flank, chest wall, back, hip and perineum. [scienceopen.com]

  The mass was subcutaneous and the margins were ill-defined. [diagnosticpathology.biomedcentral.com]

• Subcutaneous Nodule

  […] mass Classic ES Dermal or subcutaneous nodule Proximal ES Subcutaneous or deeper mass Can appear more rapidly Ulcer Nonhealing ulcer with raised margin Natural History Classic ES Persistent and multiple recurrences Successive lesions often recur and [basicmedicalkey.com]

  Schreiber Case Report 80 Downloads 32 Citations Summary Epithelioid sarcoma (ES) is a rare malignant tumour of young adults, usually presenting as a skin ulcer or subcutaneous nodule in the distal portion of the upper limb. [link.springer.com]

  Due to location, excision often has close margins, and local recurrence 10-20% Pigmented Villonodular Synovitis Synovial sarcoma Peripheral Nerve Tumors [ edit ] Neurofibroma: Small, slowly growing, cutaneous or subcutaneous nodules. May be painful. [en.wikibooks.org]

  MRI shows multiple subcutaneous nodules at the left calf below the knee integrating to a mass about 6 cm × 5 cm. [diagnosticpathology.biomedcentral.com]

  The clinical index of suspicion for a malignant lesion is often quite low. 2 In contrast, proximal-type ES presents as a nonspecific soft tissue mass. 3, 4 ES recurs in over 70% of cases, often as multiple subcutaneous nodules in the more proximal extremity [nature.com]

• Hematuria

  Presents with pain or hematuria. Does not metastasise. May be associated with tuberous sclerosis. [en.wikibooks.org]

• Hyperactivity

  Interestingly, p53 also appears to have a masked NES, which is unmasked in certain cancer cells, leading to hyperactive nuclear export and cytoplasmic accumulation of mutant protein ( Moll et al ., 1992 ; Schlamp et al ., 1997 ). [doi.org]

Initial workup for a mass that is concerning clinically for epithelioid sarcoma should be biopsied for definitive pathologic diagnosis. [statpearls.com]

During the initial workup for the left wrist lesion, a frontal chest radiograph showed no significant abnormality (Figure 4A); the patient subsequently underwent below-the-elbow amputation. [appliedradiology.com]

After a negative metastatic workup, amputation of the right hand was performed. The amputation specimen showed a tumor that extended through the entire hand with encasement of large vessels and tendons. [mdedge.com]

One should use laboratory testing in the evaluation of patients with soft tissue neoplasms as part of the general workup for systemic conditions identified in the history and physical. [podiatrytoday.com]

• Pleural Effusion

  None of the patients presented with pleural effusion. Our patient represents the first reported case of spontaneous pneumothorax and pleural effusion in both lungs in ES. [kjim.org]

  Thorascopic surgery was performed for a left pleural effusion and metastases to the left parietal pleura and adjacent soft tissue were identified. The patient was subsequently lost to follow-up. [mdedge.com]

  The actuarial 5-year distant metastasis rate was 40%, with pleuropulmonary metastases the most common site of metastatic disease, and 35% of pleuropulmonary metastases presented with pleural effusion. [thedoctorsdoctor.com]

  Ali, Metastatic proximal epithelioid sarcoma in pleural effusion: Cytopathologic findings and differential diagnosis, Diagnostic Cytopathology, 41, 6, (542-545), (2011). Ranjith Babu, Isaac O. Karikari, Thomas J. Cummings, Oren N. [doi.org]

Six patients with distant metastasis and 1 patient with an unresectable tumor received palliative treatment (30%). The remaining 16 patients underwent surgical treatment of local disease (11 patients) or locoregional disease (5 patients). [ncbi.nlm.nih.gov]

“Until today, there were no treatment options specifically for patients with epithelioid sarcoma. The approval of Tazverik provides a treatment option that specifically targets this disease. [fda.gov]

Prognosis Recurrence in 35–75%; 40–45% metastasise—e.g., to lung, regional lymph nodes, scalp; 5-year survival in 80%; 10-year survival in 50%. [medical-dictionary.thefreedictionary.com]

An aggressive treatment plan may be an option if the cell type provides a better prognosis. Find a mesothelioma specialist who is capable of treating your cell type and offering the best prognosis. [mesotheliomaguide.com]

In more specific cases also denoted that females provide superior prognosis rate than the males. The negative prognosis of the treatment and survival rate linked with the size of the tumor. [cancerwall.com]

PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSIS GENERAL Epithelioid sarcoma: the clinicopathological complexities of this rare soft tissue sarcoma. Spillane AJ, Thomas JM, Fisher C. Sarcoma Unit, Royal Marsden Hospital, London, England, UK. [thedoctorsdoctor.com]

This study is to identify the incidence of LNI, prognosis and predictors of LNI, treatment and patterns of failure. [ascopubs.org]

[…] longer recommended) Definitions Malignant mesenchymal tumor resembling carcinoma or granuloma, which shows predominantly epithelial, but also mesenchymal, differentiation ES occurs in classical and proximal (aggressive, large cell, or rhabdoid) forms ETIOLOGY [basicmedicalkey.com]

At 4.5 years, a chest CT demonstrated multiple bilateral subcentimeter, partially calcified airspace nodules of unknown etiology. Repeat CT in 3 months did not show significant interval findings. [omicsonline.org]

(Etiology) The exact cause and mechanism of Epithelioid Sarcoma formation, is unknown. [dovemed.com]

Etiology Epithelioid sarcomas are malignant tumors with mixed differentiation toward both mesenchymal and epithelial cell types. [statpearls.com]

The etiology of CIRC sarcoma remains speculative. Gould et al. [11] described an increased number of CIRCs in lymphadenopathy associated with various conditions as a result of hyperplasia-like response or CK 8/18 gene induction. [ijpmonline.org]

We sought to identify the important prognostic parameters using the Surveillance, Epidemiology, and End Results (SEER) database. [doi.org]

[…] mesenchymal, differentiation ES occurs in classical and proximal (aggressive, large cell, or rhabdoid) forms ETIOLOGY/PATHOGENESIS Genetic Factors Some cases have abnormalities of chromosome 22q Rare association with neurofibromatosis type 2 CLINICAL ISSUES Epidemiology [basicmedicalkey.com]

Upper-extremity sarcomas in the United States: Analysis of the surveillance, epidemiology, and end results database, 1973-1998. J Hand Surg Am 2003;28:511-8. 3. Jawad MU, Extein J, Min ES, Scully SP. [ccij-online.org]

As hypoxic stress contributes to many (patho)biological disorders, this new role for HIF-1alpha in hypoxic control of cell growth and death may be of general pathophysiological importance. [ncbi.nlm.nih.gov]

[…] sarcomas, [2] and have a predilection for men (up to 2:1 male to female ratio). [6] Most reported tumors occur in young males ranging in age from 10 to 45 years. [2] [3] [1] [7] The extremes of ages include ages 4 to 90, with a median age of 27 years. [6] Pathophysiology [statpearls.com]

As hypoxic stress contributes to many (patho)biological disorders 1, 5, this new role for HIF-1α in hypoxic control of cell growth and death may be of general pathophysiological importance. [doi.org]

Author information Affiliations Department of Pathology, First Affiliated Hospital and College of Basic Medical Sciences of China Medical University, 110001, Shenyang, China Chuifeng Fan, Lianhe Yang, Xuyong Lin & Enhua Wang Institute of Pathology and Pathophysiology [diagnosticpathology.biomedcentral.com]

Surgery generally involves removing the cancer and a margin of healthy tissue surrounding it to help prevent recurrence. [mayoclinic.org]

Loss of HIF-1alpha reduces hypoxia-induced expression of vascular endothelial growth factor, prevents formation of large vessels in ES-derived tumours, and impairs vascular function, resulting in hypoxic microenvironments within the tumour mass. [ncbi.nlm.nih.gov]

The TAZ–CAMTA1 fusion transcript is insensitive to the Hippo inhibitory signals that normally prevent this process and thus constitutively activates the TAZ transcriptome. [mdpi.com]

Prevention of sarcoma Sarcoma is not typically preventable. Radiation therapy is a risk factor, so reducing exposure to it may be preventive, although a need for radiation therapy usually outweighs any subsequent sarcoma risk. [medicalnewstoday.com]

Current medical research has not established a way of preventing Epithelioid Sarcoma Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are mandatory for those who have already endured ES; due to its metastasizing [dovemed.com]