长骨骨纤维结构不良型釉质上皮瘤临床病理分析

目的探讨长骨骨纤维结构不良型釉质上皮瘤的临床病理特点、鉴别诊断及生物学特征。方法对2例长骨骨纤维结构不良型釉质上皮瘤进行病理学、免疫组织化学观察,结合临床资料进行分析并复习相关文献。结果2例长骨骨纤维结构不良型釉质上皮瘤患者均为年轻人,临床以小腿疼痛或无痛性肿胀为主,病程较长,影像学均示胫骨骨干病变。光镜下肿瘤主要由梭形细胞和散在的新生编织骨组成,梭形细胞密度较高,呈束状或编织状排列,纤维组织间见少数巢团状或裂隙状的上皮样细胞巢,梭形细胞和上皮样细胞均无明显异型性。免疫表型:上皮细胞巢表达CKpan、CK14和CK19,但不表达CK8、CK18。EGFR阳性表达主要见于上皮样细胞,Ki-67在上皮样细胞和梭形细胞均有少量散在阳性表达。结论长骨骨纤维不良型釉质上皮瘤是一种罕见的好发于年轻人胫骨骨干的低度恶性肿瘤,主要根据影像学、光镜及免疫组化确诊,应与骨纤维结构不良、转移癌、滑膜肉瘤等相鉴别。     

Fine-needle aspiration of adrenal cortical carcinoma: cytologic spectrum and diagnostic challenges

We reviewed the cytologic features of 20 adrenal cortical carcinomas (ACCs; 9 primary and 11 metastatic) from 19 patients and highlighted diagnostic pitfalls. The mean size of primary ACCs was 11.9 cm, and that of metastatic ACCs was 3.0 cm. The metastatic sites were liver, lung, lymph node, soft tissue, and bone. Primary and metastatic ACCs were cytologically similar and showed a wide range of features varying from well-differentiated tumor resembling a benign cortical lesion or low-grade neuroendocrine tumor to poorly differentiated pleomorphic tumor mimicking poorly differentiated carcinoma, melanoma, or high-grade sarcoma. The common cytologic features were hypercellularity (70% of cases), necrotic debris in the background (70%), moderate to marked nuclear pleomorphism (80%), mitotic figures (90%), and prominent nucleoli (60%). Twenty percent of cases exhibited all 5 features; 40% exhibited 4 features, and 40% exhibited 3 features. Necrosis and/or mitosis were found in all cases, even in tumors with bland cytologic features. Cytologic, immunophenotypic, and ultrastructural findings should be correlated with clinical and radiologic information for achieving a proper cytologic diagnosis.     

Adamantinoma of the pretibial soft tissue. Clinicopathologic features, differential diagnosis, and possible relationship to intraosseous disease

The authors illustrate two neoplasms arising in the pretibial soft tissue that are histologically identical to adamantinoma of the long bones. The tumors developed in a 24-year-old woman and a 31-year-old man. Both were treated by local excision. Histologically, one tumor had a tubular pattern and one was composed of spindled, basaloid cells. One patient is free of disease after 14 months and one developed two recurrences after three and eight years, with the second recurrence invading the underlying tibia. Soft tissue adamantinoma may be confused with a wide range of neoplasms, including synovial sarcoma, cutaneous mixed tumor, histiocytoid hemangioma, angiosarcoma, metastatic adenocarcinoma, basal cell carcinoma, and sweat gland carcinoma. Histologic features allowing distinction from these entities and the possible relationship of soft tissue adamantinoma to its osseous counterpart are discussed.     

Ameloblastoma of the mandible: cytohistological parallels

Ameloblastoma (also known as adamantinoma, adamantine epithelioma, adamantinoblastoma, and epithelial odontoma) is a benign tumor of ectodermal origin arising from odontogenic epithelium, most commonly in the lower molar region in individuals aged between 20 and 50 years. Several histological variants are recognized, the most common being the follicular type. The present communication demonstrates the desirability of conducting parallel cytological and histological studies in cases of jaw tumors. Results of one study are reported; the cytologic profile of the tumor enabled the authors to identify cell type and establish the odontogenic origin and benign nature of the tumor. Such studies are stated to be essential in developing criteria for cytologic diagnosis of odontogenic tumors.     

Gastric carcinoma metastatic to the bone marrow: immunoperoxidase identification of KMO-1 antigen in MGG-destained aspirate.

A case is presented that illustrates the application of the immunoperoxidase technique to the May-Grünwald-Giemsa (MGG)-destained bone marrow aspirate. The cytologic findings in a MGG-stained smear of the bone marrow suggested a metastatic epithelial tumor. Subsequently, a positive reaction to KMO-1, a monoclonal antibody raised against a colon carcinoma cell line, was demonstrated in tumor cells in the MGG-destained smear sample as well as in the paraffin-embedded section of the primary gastric cancer. The demonstration of the cancer-associated antigen in the MGG-destained material may be useful in establishing the diagnosis of metastatic tumor in the bone marrow.     

Osteofibrous dysplasia and adamantinoma: correlation of proto-oncogene product and matrix protein expression

To investigate the relationship between osteofibrous dysplasia (OFD) and adamantinoma, we analyzed the expression of several proto-oncogene products and extracellular matrix proteins by immunohistochemistry and correlated our results with histological and ultrastructural findings. C-fos and c-jun, but not c-Met, were observed in OFD and in the fibrous and epithelial components of differentiated and classical adamantinomas. Staining for collagen IV, laminin and galectin-3, a laminin binding protein was seen in OFD and around cell nests in adamantinoma. E-, P-, and N-cadherin expression was found in all cases of classical adamantinoma, but not in differentiated adamantinoma or OFD. Osteonectin was detected in both the epithelial and fibrous components of adamantinomas, but osteopontin and osteocalcin were not seen in classical adamantinomas. The results show common expression of a number of oncoproteins and bone matrix proteins in adamantinoma and OFD, some of which are associated with mesenchymal-to-epithelial cell transformation. These findings would be in keeping with the hypothesis that OFD represents a precursor lesion of adamantinoma. Differential expression of a number of bone matrix protein in adamantinoma may also be of diagnostic use in distinguishing these 2 lesions immunohistochemically.     

骨肿瘤的针吸细胞学诊断

目的：探讨骨肿瘤针吸细胞学的诊断标准。方法：分析４６例骨肉瘤针吸细胞学涂片,并与２４例骨巨细胞瘤和１２例骨转移性癌进行对照,找出骨肉瘤的细胞学特征,并与组织学对照分析。结果：观察到骨肿瘤中有关１０种细胞学改变：骨肉瘤涂片中,多形性大细胞（９１．３％）,核仁明显（６９．５％）,单核瘤巨细胞（７６％）,多核瘤巨细胞（６５．２％）,核分裂象（３６．９％）,出血、坏死（９３．４％）。骨巨细胞瘤以多核巨细胞     

Adamantinoma of the tibia. An eccrine carcinoma

Recent debate concerning adamantinoma of long bone has centered around its histogenesis. The two major proposals have advocated endothelial or epithelial origin. Recent electron microscopic studies and immunoperoxidase evaluation using keratin and factor VIII-related antigen have supported an epithelial derivation. We have studied such a tumor and applied enzyme histochemical techniques in addition to previous methods. Electron microscopy disclosed desmosomes and intermediate-sized filaments. Positive carcinoembryonic antigen and keratin and negative factor VIII-related antigen confirm previous studies and support an epithelial origin. We demonstrated a correlation between enzyme staining of the tumor cells when compared with normal eccrine gland controls. Our evidence thus points to the adamantinoma of tibia as showing eccrine differentiation.     

Detection of bone marrow metastases in small cell lung cancer patients. Comparison of immunologic and morphologic methods.

An immunocytochemical method, involving four monoclonal antibodies (MAbs) previously selected for their specific binding to small cell lung cancer (SCLC) cells in human bone marrow, was used for detection of bone marrow metastases in 81 patients with diagnosed SCLC. This procedure was compared with two routine morphologic methods with regard to diagnostic efficiency and sensitivity. Bone marrow involvement was found in 26 patients (32%), one of which had limited disease according to conventional clinical criteria. Eight of the positive cases were exclusively diagnosed by immunocytochemistry, whereas the histologic and cytologic methods separately identified two patients each. Immunocytochemistry had a detection level of tumor cells in the mononuclear cell fraction of approximately 1-2%, whereas no patients with less than 10% immunocytologically detectable tumor cells were diagnosed by cytomorphologic examination of bone marrow aspirates. Evidence was obtained that the diagnostic efficiency of any method increased with the number of samples examined. Of the four MAbs used, the anti-NCAM antibody, MOC-1, labeled tumor cells in all immunologically positive patients, and in all but one of these patients all cytologically confirmed tumor cells were stained. The antibodies MOC-31, which recognize a cluster-2 antigen, and NrLu10 bound nearly all tumor cells in most cases, whereas MLuC1 only diagnosed tumor cells in a fraction of the patients. The results show that the immunocytochemical application of these antibodies is superior to morphologic techniques in detecting SCLC bone marrow metastases. Further use of the method might provide prognostically and therapeutically useful information.     

Metastatic meningioma detected in pleural fluid

Meningiomas with both malignant cytologic features and clinical behavior are rare. A 39-yr-old man with recurrent meningioma developed a pleural effusion which, on cytologic examination, contained metastatic meningioma. The diagnosis was subsequently confirmed histologically and ultrastructurally. In conclusion, metastatic meningiomas can retain meningotheliomatous cytologic features which may allow a diagnosis to be made of clinically unanticipated tumor spread. Diagn. Cytopathol. 1998;18:453–457. © 1998 Wiley-Liss, Inc.     

Papillary-cystic tumor of the pancreas in a young woman: Fine-needle aspiration cytology,ultrastructure and DNA analysis

A case of papillary-cystic tumor (PCT) of the pancreas in a young woman is reported. Fine-needle aspiration (FNA) was done preoperatively under ultrasound guidance. The aspirate showed numerous delicate papillary fragments, dyscohesive and monomorphic tumor cells with folded nuclear membranes, and foamy macro-phages. A diagnosis of PCT of the pancreas was made based on clinical, radiologic, and cytologic findings. The patient underwent distal pancreatectomy without complications. The histopathologic examination of the surgical tissue confirmed the diagnosis of PCT of the pancreas. The tumor cells were faintly positive with mucicarmine and periodic acid-schiff (PAS) stains. Immunocytochemistry using Ki67 monoclonal antibody showed a cycling index of 0.1 percent, supporting the clinical observation of low metastatic and recurrence rates of this rare tumor. DNA analysis of the tumor showed a DNA index of 1.09 (diploid) and an S-phase fraction of 5.38%. The tumor cells were positive for progestrone receptors (> 15 fmol/mg protein) but negative for estrogen receptors (< 15 fmol/mg protein). Abundant mitochondria, prominent endoplasmic reticulum and few junctional complexes were noted on electron microscopy. Emphasis is placed on accurate diagnosis based on preoperative FNA cytology in order to maximize cure rates while minimizing surgical risk and complications. © 1994 Wiley-Liss, Inc.     

Metastatic nasopharyngeal carcinoma: cytologic features of 18 cases.

The cytologic features of 18 fine-needle aspirates (FNAs) of metastatic nasopharyngeal carcinoma from 17 patients were examined. The 12 males and 5 females had a median age of 45 years (range 17-75 years). Six were white, five Oriental, four Hispanic, and two black. All patients had mid- or upper-cervical lymphadenopathy (14 bilateral, 3 unilateral). Seven developed widespread metastasis (bone, 5; lung, 2; liver 1; adrenal, 1; soft tissue, 1). The FNAs were from cervical lymph nodes (15), liver (1), adrenal (1), and soft tissue (1). Most aspirates showed similar cytologic features. Tumor cells were present singly and in syncytial groups with overlapping moderately pleomorphic oval to spindle-shaped nuclei with thin, slightly irregular nuclear contours, moderately hyperchromatic chromatin, and usually one or two prominent nucleoli. The cytoplasm was scant and pale with ill-defined borders. Mitoses were frequent. Mature lymphocytes were common in the background of lymph node aspirates. Electron microscopy and immunocytochemistry confirmed the epithelial nature of the tumor in four cases. Although the cytologic features of metastatic nasopharyngeal carcinoma (NPC) are characteristic, other poorly differentiated neoplasms need to be considered. Clinical and radiologic data are helpful in supporting the cytologic diagnosis.     

Skeletal EWSR1-NFATC2 sarcoma previously diagnosed as Ewing-like adamantinoma: A case report and literature review emphasizing its unique radiological features

Ewing-like adamantinoma (EAD) is a rare bone tumor. It remains unclear whether EAD belongs to adamantinoma, Ewing sarcoma (ES), or an independent category. Herein, we present a case of femoral sarcoma previously diagnosed as EAD in a 26-year-old woman. We observed amplified EWSR1 and NFATC2 fusion signals using fluorescence in situ hybridization. Prompted by its unique radiological features, we reviewed the current literature on skeletal EWSR1-NFATC2 sarcoma (ENS) and EAD. In addition to the similar histological features, we found that both ENS and EAD displayed similar characteristic radiological features, such as the tendency to occur in the diaphysis of long bones, cortical expansion and buttressing-type thickening, and bone surface involvement with saucer-like erosion without cortical destruction. We believe that these unique radiological features were related to its indolent behavior. Altogether, it is possible that previously reported EAD cases may be neither ES nor the classic adamantinoma but ENS. Further studies are needed to clarify the relationship between EAD and ENS.     

Adamantinoma of long bone: An analysis of nine new cases with emphasis on metastasizing lesions and fibrous dysplasia-like changes

Nine new cases of adamantinoma of long bone are presented. The age range, location, and symptomatology are similar to those in previous reports. Histologically most of the tumors have a “basaloid” or “spindled” pattern. A squamoid pattern with keratinization or an “angioblastic” pattern is seen less frequently. In all cases with adequate material there has been an associated fibrous dysplasia-like lesion. The adamantinoma is regarded as a unique neoplasm capable of differentiating along epithelial as well as mesenchymal lines. The fibrous dysplasia-like lesion is interpreted as being a part of the spectrum of mesenchymal differentiation of which the tumor is capable. Two cases in which metastasis occurred are compared to previously reported metastasizing cases. Few criteria are available that identify the potentially metastasizing tumor.     

Osteosarcoma metastatic to adrenal gland diagnosed by fine-needle aspiration

Osteosarcoma, a primary malignant tumor of the long bones, frequently metastasizes to the lungs. We report an unusual case of osteosarcoma metastatic to the right adrenal gland in a 37-yr-old male who presented 8 yr after remission with an adrenal mass. A preoperative diagnosis was made by fine-needle aspiration (FNA) biopsy. FNA biopsy revealed pleomorphic oval cells with prominent nucleoli, spindle cells, and giant tumor cells. Diagnostic osteoid was readily seen on smears and was also detected by polarization of cell-block section. Immunocytochemical stains revealed positivity of tumor cells for vimentin and osteonectin. Cytokeratin stains were negative. The cytologic diagnosis of metastatic Osteosarcoma was made, which was later confirmed upon resection of tumor by histology. Although the role of FNA in the diagnosis of primary bone tumors, including osteogenic sarcoma (OGS), remains controversial, this case, however, demonstrates the value of FNA biopsy combined with immunocytochemistry performed on the aspirated material in diagnosing osteosarcoma from an unusual location such as the adrenal gland.     

Fine-needle aspiration cytology diagnosis of metastatic nonhaematological neoplasms of the breast: a series of seven cases

Metastatic neoplasms of the breast are rare. Mammary metastases as the initial presentation are even more infrequent and can simulate a primary malignancy clinically and radiologically. Recognition of metastatic tumors in the breast is important because it would prevent unnecessary mutilating surgery and would lead to appropriate treatment of the primary tumor. There is a broad variety of cytological appearances reported about primary tumors and few reports about secondary breast malignancies, specially diagnosed by FNAC. This study was carried out to examine the clinical and cytomorphologic features of metastatic breast tumors found in 12 de Octubre University Hospital during a period of 20 years. It confirms the utility of FNAC and describes findings that can help in the differential diagnosis that sometimes can be very difficult. Seven cases of nonhematological metastatic neoplasms of the breast were identified from the files of the Department of Pathology of the 12 de Octubre University Hospital from a total of 64,000 aspirates. We included only metastatic tumors from extramammary nonhematological neoplasms. There were nine cases of hematological metastatic neoplasm that were excluded. They were diagnosed with FNAC and confirmed by histopathology, with at least three years of follow up. The breast lump was the first manifestation of malignancy in one case of synovial sarcoma. The other six cases had been previously diagnosed of cancer. These included one malignant melanoma, one alveolar rhabdomyosarcoma, one mixed müllerian tumor, one medullary carcinoma of thyroid, one colonic adenocarcinoma, and one gastric adenocarcinoma. The period of time between primary tumor and metastases ranged from one month to eight years. An accurate cytologic diagnosis was made in all the cases. Immunocytochemistry was available but diagnosis could be made with cytomorphology alone in the seven cases. Fine-needle aspiration cytology is an excellent first line diagnostic modality that is particularly informative when clinical previous data are known. If metastatic disease is suspected, the material obtained by FNAC may provide a definitive diagnosis and prevent open surgical biopsy or mastectomy. We concur with previous reports that FNAC is a reliable, rapid, secure, and cost-effective approach to the diagnosis of palpable metastatic breast tumors.     

Fine-needle aspiration biopsy of tibial adamantinoma: a case report

Adamantinoma of long bones is a rare, primary bone tumor of controversial histogenesis, usually arising in the anterior midshaft of the tibia. This slowly growing, radiolucent, expansile tumor eventually causes thinning or destruction of overlying cortical bone. Fine-needle aspiration biopsy (FNAB) is a rapid, safe, and relatively painless means of obtaining diagnostic material from bone tumors, provided the mass has eroded through or markedly attenuated the overlying cortex. A case is presented of primary adamantinoma of the tibia which was sampled first by FNAB and subsequently by surgical biopsy.     

Cytologic and immunohistochemical diagnosis of neuroendocrine (Merkel cell) carcinoma in cerebrospinal fluid

Neuroendocrine (Merkel cell) carcinoma of the skin is a rare entity. Often locally aggressive, this lesion may also metastasize to organ systems, including bone, liver, and brain. The authors report a case of a 64-year-old male who presented with hoarseness and dysphagia 17 months after resection of a primary Merkel cell carcinoma of the nose. Additional studies revealed bilateral vocal cord paralysis secondary to central nervous system dysfunction. Cytologic evaluation of the cerebrospinal fluid revealed malignant tumor cells consistent with metastatic Merkel cell carcinoma. Presented are the cytologic and immunohistochemical findings in a case of metastatic Merkel cell carcinoma involving the central nervous system.     

Fine-needle aspiration of ganglioneuroma

Ganglioneuroma is a rare soft tissue neoplasm, arising most frequently in the mediastinum or retroperitoneum in children and young adults. Correct evaluation of this lesion is of crucial importance, as there are some malignant neoplasms that have similar clinical presentations. As reports of the cytologic appearance of ganglioneuroma are few, the author describes the cytologic features of three patients examined initially by fine-needle aspiration (FNA) with correlation to histology and clinical data. This paper shows that the cytologic features of ganglioneuromas can be diagnostic if both spindle cells and ganglion cells are present in smears. The absence of immature cells and necrosis/inflammation differentiates ganglioneuromas from their malignant counterparts, ganglioneuroblastomas and neuroblastomas. Immunocytochemistry can help to establish the neurogenic origin of the tumor cells.     

Expression of growth factors and their receptors in adamantinoma of long bones and the implication for its histogenesis

Adamantinoma of long bones is a rare bone tumour with (immuno-) histological features of epithelial cells, surrounded by various amounts of osteofibrous tissue. Recent studies have indicated that cells with an epithelial phenotype are most probably the malignant element. There is still debate as to whether the fibrous part should be designated as a benign neoplastic element of a biphasic tumour or as a reactive non-neoplastic tissue next to an epithelioid bone tumour. The expression of fibroblast growth factor type 2 (FGF-2), epidermal growth factor (EGF), and their respective receptors FGFR-1 and EGFR, as well as the proliferation marker Ki-67, was studied in both constituents of adamantinoma in serial sections of 25 cases by immunohistochemistry. Expression of FGF-2 and its receptor was present in both constituents of adamantinoma, but predominated in the epithelial component. Expression of EGF and its receptor was restricted to the epithelial component of adamantinoma. Comparing osteofibrous dysplasia (OFD)-like adamantinoma with classic epithelial cell-rich adamantinoma, the expression of FGF-2, EGF, and EGFR was more intense and in a higher percentage of cells in classic adamantinoma. Proliferative activity was found nearly exclusively in the epithelial component. These data further substantiate the hypothesis that epithelial cells constitute the proliferating tumour cell population responsible for the malignant behaviour of adamantinoma. The data indicate that during progression, the epithelial cells acquire expression of FGF-2, EGF, and EGFR, accompanied by a higher proliferative activity. Within the epithelial cell population, there exists an autocrine pathway of growth stimulation. Furthermore, these data point to an interaction between the epithelial and fibrous components, in which the epithelial cells additionally stimulate fibrous cell growth via a paracrine pathway involving FGF-2. © 1998 John Wiley & Sons, Ltd.