Clear cell sarcoma like tumor of gastrointestinal tract: Experience of three cases and review of literature

Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSGT) is a rare, aggressive tumor with many histological mimickers. Herein, we have documented our experience of three cases of CCSGT and reviewed the literature. The index cases were identified in male patients in their twenties, one in jejunum and two in the distal colon. Histomorphological examination revealed the characteristic heterogeneous histomorphology with patchy immunohistochemical positivity with S100 protein and negative melanocytic markers. The fluorescence in-situ hybridization test showed translocation of the EWSR1 (22q12) gene in >80% tumor cells. While one of our patients died after 2 years with lung metastasis, the other two patients are still alive on 1.5 years and 3 months follow up, respectively. CCSGT is a rare malignant tumor of the gastrointestinal tract. Although characteristic morphology, use of a judicial panel of immunohistochemical stains, and translocation study for EWSR1 gene can establish the diagnosis, experience in adjuvant therapy is still limited.

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.

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.

Clear cell sarcoma of gastrointestinal tract：clinicopathologic analyses and review of litera-tures / 临床与实验病理学杂志

Purpose To study the clinicopathologic and genetic features of clear cell sarcoma of the gastrointestinal tract. Methods One case of clear cell sarcoma of the gastrointestinal tract was analyzed by histology, immunohistochemistry, fluorescence in situ hy-bridization ( FISH) , with review of relevant literatures. Results A female patient was admitted to the hospital with a history of 1-week abdominal pain. Computed tomography revealed a mass in right hemicolon. A grey-white ulcerated mass was observed in the colon, in-volving the entire thickness of the colon wall extending into the subserosa. Microscopically, medium-size oval or round cells were ar-ranged in sheets and scattered osteoclast-like multinucleated giant cells were also presented. Immunohistochemically, the tumor cells were strongly positive for S-100 protein, but negative for HMB-45, Melan-A, CD117, CD1a and PCK. FISH showed the rearrangement of EWSR1 in 74% of the neoplasm cells. Conclusions Clear cell sarcoma of the gastrointestinal tract is an extraordinarily rare gastro-intestinal neoplasm that shows peculiar histopathological, immunohistochemical, ultrastructural, and genetic features. Whether clear cell sarcoma-like gastrointestinal tumor represents a distinct entity awaits larger series, including cytogenetic or molecular biological in-vestigation.

A Case of Mucinous Cystadenoma with Sarcoma-Like Mural Nodule / 대한산부인과학회잡지

Mural nodules in mucinous ovarian tumors are very rare. Since Prat and Scully first described sarcomatous and sarcoma-like mural nodules within mucinous ovarian tumors in 1979, only about 30 cases have been reported in the literature. Pathologically, mural nodules have three different types; i.e. 1) sarcoma-like mural nodule, 2) sarcomatous mural nodule, 3) anaplastic carcinomatous mural nodule. It is important to distinguish sarcoma-like mural nodule from another two type. because the former in the mucinous ovarian tumor has more favorable prognosis than the latter. We report a case of mucinous cystadenoma with sarcoma-like mural nodules in a 23-year-old nulliparous woman.

A Case of Ovarian Mucinous Cystadenocarcinoma with Mural Nodule of Anaplastic Carcinoma / 대한부인종양콜포스코피학회잡지

Mural nodules in ovarian mucinous tumors, whether benign, borderline, or malignant, have been described by several authors since Prat and Scully first described sarcoma or sarcoma-like mural nodules occurring in association with mucinous epithelial neoplasia in 1979. Three distinct types of the mural nodule have been identified; i. e., 1) sarcoma-like lesion, 2) true mesenchymal sarcome, and 3) anaplastic carcinoma simulating a sarcoma. Mural nodules of sarcomatous and carcinomatous foci associated with mucinous ovarian tumors should be separated from sarcoma-like nodules because of the poor prognosis of the former compared to the favorable prognosis of the latter. We experienced a case of ovarian mucinous cystadenocarcinoma with sarcoma-appearing mural nodule of anaplastic carcinoma occurred in a 59-year-old woman and reported with brief review of the literatures.

Sarcoma-like Mural Nodule in Ovarian Mucinous Cystadenocarcinoma: A case report

We describe an ovarian mucinous cystadenocarcinoma with a sarcoma-like mural nodule. In the literature, rare cases of ovarian mucinous tumors have been described which contain foci of undifferentiated carcinoma, sarcoma, and sarcoma-like nodules. The distinction between these lesions is important because of poorer prognosis of true sarcoma and anaplastic carcinoma than sarcoma-like mural nodules. This case shows different results of immunohistochemical stain for anaplastic carcinoma.