Chondroid chordoma: fine-needle aspiration cytology with histopathological, immunohistochemical, and ultrastructural study of two cases.

Chondroid chordoma is a controversial and confusing entity that was originally described by Heffelfinger et al. (Cancer 1973; 32:410-420) as a biphasic malignant neoplasm possessing elements of both chordoma and cartilaginous tissue. Fine-needle aspiration (FNA) cytology of chondroid chordoma has not been described. The aim of our investigation was to characterize the chondroid area of chondroid chordoma and to compare the FNA features with those of well-differentiated chondrosarcoma. Clival and cervical spine chondroid chordomas were studied with light microscopy, immunohistochemistry, and electron microscopy. Chondroid chordomas demonstrated an epithelial nature by immunohistochemistry and ultrastructural studies. The FNA smears showed low cellularity, with loosely arranged or dispersed round cells in a myxoid background. Although the smears were similar to those of well-differentiated chondrosarcomas, they showed a positive reaction for epithelial markers. These findings reveal that chondroid chordoma is a variant of chordoma which possesses a hyaline matrix. Immunohistochemical demonstration of epithelial markers is useful to distinguish it from chondrosarcoma. Diagn. Cytopathol. 1999; 21:335-339.     

Logistic regression analysis of myxoid sarcomas: A cytologic study

The objective of this study was to identify key diagnostic cytologic criteria for the most common myxoid sarcomas studied by fine-needle aspiration cytology. We reviewed 27 myxoid malignant fibrous histiocytomas, 8 chordomas, 16 chondrosarcomas, and 12 myxoid liposarcomas in which both cytologic specimens and final histopathologic diagnoses were available. All specimens were coded as to the presence or absence of the following variables: high cellularity, low cellularity, tissue fragments, epithelial fragments, pale/ loose ground substance, dense ground substance, chondroid fragments, large amount of myxoid material, small amount of myxoid material, capillary vessel networks, pleomorphism, binucleate cells, multinucleate cells, physaliphorous cells, cells in lacunae, signet ring cells, lipoblasts, fibroblast-like cells, histiocyte-like cells, stellate cells, long filamentous cells, short spindle cells, osteoclastic giant cells, nuclei with pointed ends, nuclei with cigar-shaped ends, fish-hook nuclei, round/ ovoid nuclei, naked nuclei, large nucleoli, small nucleoli, mitotic figures, abnormal mitotic figures, intracytoplasmic hemosiderin deposits, background cells, fat, cytoplasmic vacuoles, and pleomorphic giant cells. A logistic regression analysis was performed to identify the variables predictive of myxoid malignant fibrous histiocytoma, chordoma, myxoid chondrosarcoma, and myxoid liposarcoma. The statistical analysis selected pleomorphic giant cells and the presence of fibroblast-like cells as most predictive of malignant fibrous histiocytoma, physaliphorous cells as most closely associated with chordoma, chondroid fragments as most predictive of chondrosarcoma, and lipoblasts as most predictive of liposarcoma. While myxoid lesions have many overlapping cytologic features, key criteria including the presence of lipoblasts, physaliphorous cells, chondroid fragments, and pleomorphic giant cells are useful in subclassifying these neoplasms. Diagn. Cytopathol. 1998;19:355–360. © 1998 Wiley-Liss, Inc.     

Chordomalike Soft Tissue Sarcoma in the Leg: A Light and Electron Microscopic and Immunohistochemical Study

A soft tissue tumor in the leg of a 67-year-old woman is described. This large tumor below the knee area infiltrated extensively the deep and superficial soft tissues but did not involve the bones. The tumor cells formed nodules resembling the architecture seen in chondroid tumors and chordoma. The tumor cells were often vacuolized, and there was extracellular myxoid matrix similar to that in chordoma or myxoid chondrosarcoma. Immunohistochemistry showed keratins 8 and 19, epithelial membrane antigen, and vimentin in most tumor cells, and there was also S-100 protein positivity in a number of tumor cells. Electron microscopy showed desmosomelike cell junctions and bundles of intermediate filaments resembling those seen in many epithelial neoplasms. Thus the tumor resembled chordoma in many respects. Because clinically no other primary tumor was found, this tumor is probably a chordomalike primary soft tissue sarcoma different from typical extraskeletal myxoid chondrosarcoma or chordoid sarcoma.     

Chordomalike Soft Tissue Sarcoma in the Leg: A Light and Electron Microscopic and Immunohistochemical Study

A soft tissue tumor in the leg of a 67-year-old woman is described. This large tumor below the knee area infiltrated extensively the deep and superficial soft tissues but did not involve the bones. The tumor cells formed nodules resembling the architecture seen in chondroid tumors and chordoma. The tumor cells were often vacuolized, and there was extracellular myxoid matrix similar to that in chordoma or myxoid chondrosarcoma. Immunohistochemistry showed keratins 8 and 19, epithelial membrane antigen, and vimentin in most tumor cells, and there was also S-100 protein positivity in a number of tumor cells. Electron microscopy showed desmosomelike cell junctions and bundles of intermediate filaments resembling those seen in many epithelial neoplasms. Thus the tumor resembled chordoma in many respects. Because clinically no other primary tumor was found, this tumor is probably a chordomalike primary soft tissue sarcoma different from typical extraskeletal myxoid chondrosarcoma or chordoid sarcoma.     

Cytologic findings of myxoid neurothekeoma: case report based on fine-needle aspiration cytology,immunohistochemistry, and correlating histopathology

Myxoid neurothekeomas (nerve sheath myxomas) are rare benign cutaneous neoplasms that may morphologically mimic other myxoid neoplasms of skin and soft tissue. The cytologic and histopathologic features of this lesion may resemble various myxoid sarcomas, chordoma, myxoid neurofibroma, dermal cutaneous mucinosis, and cutaneous myxoma as well as other myxoid or chondroid neoplasms. In this study, a myxoid neurothekeoma was analyzed using multiple techniques. We found that myxoid neurothekeomas reveal a nonspecific pattern by fine-needle aspiration, including stellate cells embedded within an abundant metachromatic myxoid stromal matrix. These are cytologic features shared by various other subcutaneous neoplasms and thus may not be helpful in forming a definitive diagnosis. Histopathologically, the tumor is composed of nodules of myxoid stroma containing interspersed bland spindled and stellate cells. Immunohistochemical studies show tumor cell positivity for S-100 protein and vimentin, a profile shared by other neoplasms with similar cytologic features and therefore of little diagnostic value. The histologic and cytologic differential of subcutaneous and soft tissue myxomatous lesions is broad and, therefore, is of unique value to the cytopathologist to consider myxoid neurothekeomas among the differential of other myxomatous neoplasms.     

骨外黏液样软骨肉瘤2例报道及文献复习

目的探讨骨外黏液样软骨肉瘤的临床病理学特征。方法对2例骨外黏液样软骨肉瘤进行光镜观察及免疫组化染色标记,并通过相关文献复习,对病理诊断及鉴别诊断等指标进行分析。结果1例发生于足底,1例发生于乳腺。光镜下肿瘤呈分叶状,边界清。细胞为圆形及短梭形,成束状排列于黏液样基质中,局部可见围血管形成玫瑰花结样结构。部分肿瘤细胞异型性明显。免疫表型:vimentin、NSE、Syn均呈阳性;例1EMA灶性阳性,例2阴性;S-100蛋白、CgA及CK均阴性。结论骨外黏液样软骨肉瘤为罕见的软组织恶性肿瘤,具特异性的组织病理学特点。主要发生于四肢,少数可发生于实质器官,至今未有乳腺原发病例报道。部分肿瘤细胞可发生间变导致诊断困难,须与脊索瘤、骨内软骨肉瘤、化生性癌及黏液性肿瘤等鉴别。     

Fine-needle aspiration cytology findings in a case of aggressive angiomyxoma: A case report and review of the literature

Aggressive angiomyxomas are uncommon but distinct soft-tissue neoplasms occurring predominantly in the pelvis and peritoneum of females, but they have occasionally been reported in association with inguinal hernias in males. Histologically, these neoplasms are characterized by a proliferation of spindle- or stellate-shaped cells widely separated by loose myxoid stroma in which is dispersed a prominent vascular component. The vascular component is comprised of large, thick-walled vessels that generally do not show an arborizing pattern. Mitotic activity has been exceedingly low in the cases reported. Because of their occurrence within the groin, these lesions may undergo fine-needle aspiration (FNA). Cytologic examination of this material will reveal hypocellular smears containing scattered spindle cells with bipolar cytoplasmic processes, as well as bland stellate cells. The nuclei are fusiform to oval with a bland chromatin pattern. The stromal cells lie in a background of watery myxoid material. While specific diagnosis by FNA is not possible, the recognition of this cytologic appearance should exclude lymphoproliferative processes as well as metastatic disease from the differential diagnosis. Careful attention to cytologic detail should also help exclude certain other myxoid neoplasms, especially myxoid liposarcoma. Once the myxoid stromal nature of the proliferation is recognized, a differential diagnosis of myxoid lesions can be considered along with a recommendation for open biopsy to establish the definitive diagnosis. Diagn. Cytopathol. 16:425–429, 1997. © 1997 Wiley-Liss, Inc.     

Parapharyngeal chordoma: A diagnostic challenge and potential mimic of pleomorphic adenoma on fine‐needle aspiration cytology

Chordomas are rare tumors that are usually located in the sacrococcygeal and sphenooccipital region. Their cytologic diagnosis is rather straightforward when sampled by fine‐needle aspiration (FNA) from these characteristic locations, especially when physalipherous cells are present. However, chordomas may pose difficult diagnostic challenges when encountered in unusual locations, such as the parapharyngeal region. We report the cytologic findings of a recurrent chordoma sampled through transoral FNA from the parapharyngeal space of a 66‐year‐old woman. As the prior history of chordoma was not available during the rapid onsite evaluation, the presence of bland epithelioid nonvacuolated cells and spindle cells intimately admixed with a fibrillary, intensely metachromatic material led to an initial diagnosis of pleomorphic adenoma. Review of the patient's prior pathology specimen and of the Papanicolaou‐stained smears and cellblock sections showing rare multivacuolated (physalipherous) cells led to the correct diagnosis, which was supported by immunoperoxidase stains (cytokeratin AE1/AE3+, S100+, GFAP?). A review of the literature found no previous instances in which chordomas mimicked pleomorphic adenoma on FNA. However, since the two tumors show significant cytomorphologic overlap, including the presence of abundant fibrillary matrix with embedded neoplastic cells and single bland spindle and epithelioid tumor cells with occasional intranuclear pseudoinclusions, we compared their cytologic features. A review of the FNA cytologic features of this case of chordoma and of 17 consecutive cases of pleomorphic adenoma found that the presence of a more abundant, focally vacuolated cytoplasm favors chordoma over pleomorphic adenoma. Diagn. Cytopathol. 2013. © 2011 Wiley Periodicals, Inc.     

Myxoid chondrosarcoma of the sphenoid sinus and chondromyxoid fibroma of the iliac bone: cytomorphologic findings of two distinct and uncommon myxoid lesions

Myxoid chondrosarcoma (MCS) and chondromyxoid fibroma (CMF) are two uncommon myxoid cartilaginous neoplasms with distinct cytologic features, histologic patterns, and immunoprofiles. Because these neoplasms have characteristic biological behaviors and management, their correct diagnosis is crucial to avoid debilitating and unnecessary surgical procedures. We report the imprint cytology (IC) preparation findings along with the differential diagnosis in one case each of myxoid chondrosarcoma and chondromyxoid fibroma of the splenoid sinus and iliac bone, respectively. The two great mimickers for these neoplasms, chordoma and chondrosarcoma, represent difficult diagnostic challenges, especially when MCS and CMF occur in unusual locations. IC in conjunction with the clinical and radiologic findings can provide a rapid preliminary intraoperative diagnostic interpretation which can aid in planning the immediate surgical management, as well as guide specific tissue triage for key ancillary studies such as electron microscopy and cytogenetic analyses. To the best of our knowledge, there have been no cytologic reports of MCS of the sphenoid sinus and CMF of the iliac bone.     

Cytopathological dilemma of anaplastic sacral chordoma with radiological and histological corroboration

Chordoma is a relatively rare locally invasive and potentially malignant tumor of fetal notochord origin, affecting the axial skeleton. Cytopathological diagnosis of chordoma is favored by the presence of characteristic physaliphorous cells, bearing abundant foamy cytoplasm dispersed in a myxoid matrix. Anaplastic chordoma or dedifferentiated chordoma, an even rarer variant, can cause a diagnostic confusion with chondrosarcoma from the cytopathological point of view, with similar chondromyxoid matrix and atypical cells. Hence, chordoma bearing anaplastic features needs to be identified and should be distinguished from chondrosarcoma on aspiration cytopathology. We present a case of anaplastic sacral chordoma in a man 59 years of age, causing extensive destruction of sacrum and invading the paravertebral tissues as evidenced by radiology. Fine needle aspiration cytopathology revealed few large pleomorphic hyperchromatic cells, admixed with characteristic physaliphorous cells and myxoid matrix. The cytopathological diagnosis has been confirmed by histopathology and immunohistochemistry. Since anaplastic chordoma bears an unfavorable prognosis, it should be suspected on preoperative aspiration cytopathology. Clinicoradiological correlation along with histopathological and immunohistochemical confirmation is necessary subsequently.     

Cytopathologic characteristics and differential diagnostic considerations of osteolytic myxopapillary ependymoma

Myxopapillary ependymoma (MPE) is a rare variant of conventional ependymoma found predominantly in the sacrococcygeal region in young adults and characterized by its distinct epithelial and stromal components (WHO grade I designation). MPE with extensive osteolysis is extremely uncommon and only up to 40 cases have been documented. A case is presented here in which imprint smears of a sacral tumor in an 18‐year‐old man revealed complex papillary structures, small loose clusters, or cord‐like structures of bland tumor cells embedded in a myxoid or mucinous background. The tumor cells possessed uniformly round nuclei with a smooth nuclear outline, fine granular chromatin, and small nucleoli. Slender cytoplasmic fibrillary processes and occasional intracytoplasmic vacuoles were observed. A cytologic diagnosis of a MPE was suggested and histochemical and immunohistochemical studies were conducted on formalin‐fixed, paraffin‐embedded material. Immunohistochemically, the tumor cells showed diffuse and strong membranous and cytoplasmic staining for cytokeratin AE1/AE3, glial fibrillary protein, and S‐100 protein, but negative for epithelial membrane antigen, pan‐neuroendocrine markers (i.e., NSE, chromogranin A, synaptophysin), or brachyury. The proliferative index with MIB‐1 was around 10%. The diagnosis of osteolytic MPE was confirmed based on cytopathologic, histopathological, immunohistochemical results, radiologic findings, and the location of the tumor. We demonstrated here the cytopathological features of osteolytic MPE with emphasis on differential diagnostic considerations. Diagn. Cytopathol. 2014;42:778–783. © 2013 Wiley Periodicals, Inc.     

Light and electron microscopic examination of fine-needle aspirates in the preoperative diagnosis of cartilaginous tumors

Twenty-eight patients with chondrogenic tumours--2 chondroblastomas, 4 chondromas, 18 chondrosarcomas, 1 clear-cell chondrosarcoma, and 3 mesenchymal chondrosarcomas--underwent fine-needle aspiration biopsy (FNAB) in the preoperative investigation. The cytologic features in smears were compared with the histopathologic findings in the surgical specimens; in 14 cases they were also compared with the light and electron microscopic findings in resin-embedded fine-needle aspirates. The smears of the vast majority of the classical chondrosarcomas presented features that made possible the FNAB diagnosis of a chondrogenic tumor to be made. In the case of the low-grade chondrosarcomas in particular, which were poorly or moderately cellular in smears and showed chondroblastic cells often in lacunary structures of hyaline matrix, consideration of the clinical presentation, size, location, and roentgenographic appearance was essential for the diagnosis of chondrosarcoma. On the other hand, the high-grade chondrosarcomas presented cytologic features that clearly indicated their malignancy and they usually had a myxoid matrix. The possible differential diagnoses that may arise from the FNAB diagnosis of cartilaginous tumors are discussed. The resin-embedding technique for the light and electron microscopic examination of FNABs, along with the histochemical analysis for the demonstration of sulphated glucosaminoglycans and the immunocytochemistry applied to smears, was found to be of value in the definite diagnosis, especially in the distinction of chondrogenic tumors from chordoma and metastatic mucous-producing carcinoma.     

Endoscopic ultrasound-guided fine-needle aspiration cytology diagnosis of solid-pseudopapillary tumor of the pancreas: a rare neoplasm of elusive origin but characteristic cytomorphologic features

Clinical histories, endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) material, and immunohistochemical stains performed on cell block samples of 6 solid-pseudopapillary tumors of the pancreas (SPTPs) were reviewed in the cases of 5 females (13-58 years) and 1 man (57 years); all had abdominal pain. Preliminary cytologic diagnoses at endoscopy included 1 SPTP 2 low-grade neoplasms, and 3 pancreatic endocrine tumors. Variable numbers of branching fragments with central capillaries and myxoid stroma were seen in the smears of 5 of 6 cases but were more apparent in the cell block material of all cases. The cells had bland nuclear features and rare grooves. Extensive necrosis was noted in 1 case and rare mitotic figures in 1. SPTPs showed strong cellular immunoreactivity for vimentin and focal weak keratin reactivity. Neuron-specific enolase, alpha1-antitrypsin, and alpha1-antichymotrypsin stains performed in 2 cases were strongly positive. Subsequent surgical resection confirmed all diagnoses. EUS-guided FNA diagnosis of SPTP is accurate. The characteristic branching papillae with myxoid stroma are best seen in cell block slides. Clinical setting, cytomorphologic features, and immunostains of the cell block help distinguish SPTP from pancreatic endocrine tumors, acinar cell carcinoma, and papillary mucinous carcinoma.     

Metastatic myxopapillary ependymoma: Report of a case with fine-needle aspiration findings

A case of recurrent and metastasizing myxopapillary ependymoma of the sacral region in a 35-yr-old man is reported. Fifteen years after the original diagnosis, he presented with an abdominal mass, subcutaneous nodules in the previous surgical excirion line, and bilateral inguinal lymph node enlargement. FNA cytology of the inguinal lymph nodes showed a poorly cohesive, highly cellular smear pattern exhibiting papillary formations and rosette-like structures composed of slim columnar cells having cytoplasmic processes without evidence of atypia. Histologic and ultrastructural findings confirmed the diagnosis. Chromosomal analysis was also done. © 1994 Wiley-Liss, Inc.     

Chondroid chordoma of the skull base diagnosed by squash-smear technique

Squash-smears of tissue from a chondroid chordoma arising from the skull base of a 26-year-old white man showed numerous single and clustered physaliferous cells with multiple intracytoplasmic vacuoles, and atypical cartilaginous cells in an amorphous myxoid background.     

Expression of tau proteins and tubulin in extraskeletal myxoid chondrosarcoma, chordoma, and other chondroid tumors.

Tau proteins are microtubule-associated proteins required for the polymerization of tubulin. The abnormal accumulation of tau proteins in neurofibrillary tangles is a well-known phenomenon and has been studied extensively. However, the role of tau protein in chondroid tissue and its neoplasia is unknown. In the present study, 2 extraskeletal myxoid chondrosarcomas (EMCs), 6 chordomas, 6 chondrosarcomas, 3 myxoid chondrosarcomas of bone, 2 osteochondromas, 6 chondroblastomas, and 2 nonneoplastic adult articular cartilages were immunostained with monoclonal antibodies against tau proteins and tubulin. The results showed that the coexpression of tau proteins and tubulin was present only in EMCs (2/2) and chordomas (4/6). Although tubulin was detected in chondroblastomas (5/6), osteochondromas (2/2), chondrosarcomas (5/6), and myxoid-chondrosarcomas of bone (3/3), tau expression was absent in these tumors. The perichondrial chondroblasts but not chondrocytes from nonneoplastic articular cartilage also localize tau and tubulin with a much weaker staining intensity. The results mainly demonstrate that there is frequent expression of tau proteins in some microtubule-rich neoplasms, such as EMC and chordoma. The different immunostaining pattern of tau proteins between chordoma and myxoid chondrosarcoma of bone may be useful in the differential diagnosis, especially when both neoplasms occur in the base of skull.     

Fine‐needle aspiration of soft tissue myoepithelioma

Soft tissue myoepithelioma is a rare neoplasm composed of myoepithelial cells. We describe the cytologic features of a soft tissue myoepithelioma arising in the right lower chest wall in a 65‐year‐old woman. The fine‐needle aspiration (FNA) smears showed round to oval, spindle, epithelioid, and plasmacytoid cells in the myxoid background. The nuclei were uniform, round to ovoid, with finely distributed chromatin and eosinophilic or pale cytoplasm, and resembled lobular carcinoma of breast. Ultrasound guided core biopsy showed the tumor cells had bland cytologic features, arranged in small cords, nests, and dissociated single cells, with no glandular differentiation or breast tissue seen. The tumor cells demonstrated immunoreactivity for cytokeratin (AE1/AE3) and glial fibrillary acidic protein, but were negative for estrogen receptor. Fluorescence in situ hybridization demonstrated the EWSR1 rearrangement, confirming the diagnosis of myoepithelioma. Diagn. Cytopathol. 2016;44:152–155. © 2015 Wiley Periodicals, Inc.     

Ciliated foregut cyst of pancreas: cytologic findings on endoscopic ultrasound-guided fine-needle aspiration

The cytologic findings of a ciliated foregut cyst of the pancreas diagnosed by endoscopic ultrasound-guided fine-needle aspiration (FNA) are described. Cytologic features of ciliated foregut cysts include the presence of ciliated columnar cells and detached ciliary tufts in a cystic fluid background with amorphous debris and rare macrophages. These cytologic findings are clearly distinct from those of cystic mucinous neoplasms and other pancreatic cysts with which the ciliated foregut cyst may be confused. To the best of our knowledge, this is the first case reporting the cytologic findings of a pancreatic ciliated foregut cyst sampled by endoscopic ultrasound-guided FNA. We believe that the distinctive and characteristic cytologic features can allow a preoperative cytologic diagnosis of this highly unusual pancreatic cystic lesion.     

Fine-needle aspiration cytopathology of soft tissue: Chondromyxoid and myxoid lesions

Soft-tissue lesions pose an intimidating challenge to diagnostic interpretation by fine-needle aspiration cytology. to better characterize such aspirates, we investigated a subgroup of lesions which yielded a large amount of chondromyxoid and myxoid stroma (CMS). Thirty-three cases (22 malignant lesions, 11 benign lesions) in which CMS was a prominent feature of the smears were studied. the most common malignant neoplasms were extraskeletal chondrosarcoma (6 cases), myxoid liposarcoma (6), and malignant fibrous histiocytoma (5). the benign entities consisted of ganglion cyst (4), myxoma (3), and neurofibroma (2). of the aspirates from 22 malignant neoplasms, 15 (68%) contained opaque stroma, and in 18 (81%) the stroma was distributed as discrete clumps or fragments; all extraskeletal chondrosarcomas and chordomas contained these features. Additionally, in five of six chondrosarcomas the matrix exhibited cells within lacunae. Except for a tracheal chondroma which extended into the adjacent soft tissue, no benign aspirates had all three of these cytologic features. Twenty-three aspirates (70%) were moderately or highly cellular; all but two of these were malignant. Moderate to marked nuclear atypia occurred in 13/22 (59%) malignant and 1/11 (9.0%) benign aspirates. With a few exceptions, aspiration smears of benign lesions with abundant CMS were characterized by low cellularity, semitransparent amorphous stroma, and lack of nuclear irregularity. Recognition of variations in the character of the background material in addition to traditional cytologic criteria can aid in the diagnosis of benign and malignant chondromyxoid and myxoid aspirates of soft tissue. © 1995 Wiley- Liss, Inc.