Exfoliative cytopathology of alveolar rhabdomyosarcoma

Exfoliative cytopathology of alveolar rhabdomyosarcoma is presented. A 34-year-old male was found to have alveolar rhabdomyosarcoma of the retroperitoneum at autopsy. Ascites was recognized in the course of this disease. Cytological features in the ascites were characterized by relatively small-sized ovoid cells observed singly or in loose clusters. The neoplastic cells possessed small amount of rather vesicular cytoplasm and an eccentric nucleus. The nucleus characteristically revealed multiple deep indentations in the nuclear membrane, a few prominent eosinophilic nucleoli and finely granular or evenly distributed chromatin pattern. These cytological findings, especially the nuclear features, might be useful to differentiate alveolar rhabdomyosarcoma from other neoplasms which consist of relatively small-sized ovoid cells even if the striated muscle origin cannot be confirmed by immunoperoxidase staining.     

Rhabdomyosarcoma in middle-aged and elderly individuals

In a review of a national series of malignant tumors in middle-aged and elderly individuals (over 40 years of age), in all 107 cases primarily diagnosed and reported to the Swedish Cancer Registry as rhabdomyosarcomas during the period 1972-1981, 4 cases were accepted as botryoid, embryonal or alveolar rhabdomyosarcoma, using light-microscopic criteria for the diagnosis. An electron-microscopic and immunohistochemical analysis was performed on the 4 cases along with 7 cases of botryoid, embryonal and alveolar rhabdomyosarcoma in patients of over 40 years of age obtained from our own files. Rhabdomyoblastic differentiation was established ultrastructurally by the presence of myofilaments and Z-like densities in 10 of these 11 cases. There were tumor cells in the formaldehyde-fixed, paraffin-embedded material which were positively stained for desmin in all cases, for myoglobin in 7/11 cases, for vimentin in 5/11 cases and for actin in all cases, using monoclonal antibodies. The demonstration of desmin by the monoclonal antibody which was used on the formaldehyde-fixed, paraffin-embedded material is of particular value in the diagnosis of rhabdomyosarcoma. Another tumor, located in the minor pelvis, lacked the light-microscopic features of botryoid, embryonal or alveolar rhabdomyosarcoma, but presented ultrastructural and immunohistochemical evidence of a rhabdomyoblastic differentiation. This tumor was epithelioid in appearance and shared features with alveolar soft part sarcoma. The label epithelioid rhabdomyosarcoma is proposed for this tumor. Nine pleomorphic sarcomas were selected from the national series as possible pleomorphic rhabdomyosarcomas because of the presence of ribbon-shaped tumor cells with an eosiniphilic cytoplasm. There was no electron-microscopic or immunohistochemical evidence of a myogenic differentiation in any of these 9 tumors. The present investigation indicates that a pleomorphic type of rhabdomyosarcoma, indistinguishable from embryonal, botryoid and alveolar rhabdomyosarcoma, is extremely rare or non-existent.     

Alveolar rhabdomyosarcoma infiltrating bone marrow at presentation: the value to diagnosis of bone marrow trephine biopsy specimens.

AIMS: To describe the histological appearances of bone marrow infiltrated with rhabdomyosarcoma at presentation and to determine their value in establishing the diagnosis. METHODS: Patients presenting over seven years in the northern health region of England with rhabdomyosarcoma were studied. Bone marrow aspirates and trephine biopsy specimens taken at presentation were examined. RESULTS: Seven of 32 patients with rhabdomyosarcoma had bone marrow infiltration, resulting in marrow failure in all cases, at diagnosis. The diagnosis was established in these seven by the typical cytological appearances and immunophenotype of the infiltrating cells (all seven patients) and cytogenetic abnormalities (three patients). Histological examination of the bone marrow showed a pseudoalveolar pattern with fibrous septal bands, enlarged vascular channels, and lack of cohesion of the tumour cells within the subdivided aggregates in all seven. In four cases multinucleate giant cells, often with peripherally sited nuclei, were found. CONCLUSIONS: These histological features of infiltrated marrow are so characteristic that the diagnosis of alveolar rhabdomyosarcoma can be made, or at least suspected, in many cases even without recourse to technically difficult and expensive further investigations. Bone marrow biopsy should be a routine part of the investigation of patients with bone marrow failure and will be of particular value in the diagnosis of those with disseminated alveolar rhabdomyosarcoma.     

Synaptic organization of the cat entopeduncular nucleus with special reference to the relationship between the afferents to entopedunculothalamic projection neurons: An electron microscope study by a combined degeneration and horseradish peroxidase tracing technique

The synaptic organization of the feline entopeduncular nucleus was studied electron microscopically. After horseradish peroxidase injections into the ventral anterior and ventral lateral nuclear complex of the thalamus, normal axon terminals synapsing with entopedunculothalamic projection neurons were classified into four types on the basis of the size and shape of synaptic vesicles in them, and types of the postsynaptic membrane differentiation. Type I and type II axon terminals were characterized by symmetrical synaptic contacts, and large ovoid or small ovoid synaptic vesicles, respectively. Type II axon terminals were further classified into two subtypes as to their sizes: one was small (IIa), the other large (IIb). Type III and type IV axon terminals were characterized by asymmetrical synaptic contacts, and large ovoid or small ovoid synaptic vesicles, respectively.

To determine the origin of each type of terminal, electrolytic lesions of the caudate nucleus or the subthalamic nuclear region were combined with horseradish peroxidase injections into the thalamus or the subthalamic nuclear region. After electrolytic lesions of the caudate nucleus, degeneration was seen in type I axon terminals contacting entopedunculothalamic projection neurons. Following electrolysis or horseradish peroxidase injection into the subthalamic nuclear region, type IIa and type IV axon terminals showed degenerations or horseradish peroxidase labelling. Such terminals also synapsed with entopedunculothalamic projection neurons. It was demonstrated that these projection neurons relay the striatal or subthalamic inputs directly to the thalamus. After horseradish peroxidase injection into the thalamus, many labelled type II axon terminals were observed to synapse with entopedunculothalamic projection neurons. Type III axon terminals were left unchanged throughout these experiments. In addition, the entopeduncular neuron was observed to receive convergent inputs from both the caudate nucleus and probably the subthalamic nucleus. Axoaxonal synapses were also found to be involved in the synaptic triad.

These results indicate that type I axon terminals originate from the caudate nucleus, part of type IIa and type IV axon terminals originate from the subthalamic nucleus or caudal to the subthalamic nuclear region, and part of type IIa and type IIb terminals come from intrinsic axon collaterals.     

Fine-needle aspiration biopsy diagnosis of rhabdomyosarcoma: cytologic, histologic, and ultrastructural correlations.

A series of 15 cases of rhabdomyosarcoma diagnosed by fine-needle aspiration biopsy (FNAB) and confirmed by histopathology is reviewed. Cytologically, the tumors were composed of a variable mixture of cells, which according to the degree of differentiation were categorized as early, intermediate, or late rhabdomyoblasts. Histologically, the tumors were divided into embryonal 9, monomorphic round cell 4, and alveolar rhabdomyosarcoma 2. Comparison of histological and cytological features revealed that embryonal types were composed mainly of early rhabdomyoblasts. Recognition of these patterns may be helpful in FNAB diagnosis of rhabdomyosarcoma.     

Fine‐needle aspiration cytology of abdominal wall endometriosis

While about 15% of menstruating women develop endometriosis, abdominal wall endometriosis is relatively rare. We present 10 cases of abdominal wall endometriosis diagnosed by fine‐needle aspiration (FNA) cytology and confirmed by subsequent surgical excision. A palpable abdominal wall mass was the most common symptom, followed by pain. Nine cases were associated with previous surgery. The smear showed glandular epithelial cells and spindle or ovoid stromal cells accompanied by macrophages in eight cases. The nucleus was round‐to‐oval and the nucleolus was inconspicuous. Mitosis was not observed in any case. In two cases, which were suspicious of malignancy on FNA, the epithelial cells showed anisonucleosis, hyperchromasia, and small conspicuous nucleoli. The stromal cells were spindle or ovoid in shape, without metaplastic changes. Histiocytes were noted in nine cases and hemosiderin‐laden histiocytes were noted in two cases. The proportion of inflammatory cells varied. FNA diagnosis of abdominal wall endometriosis is possible when the cytological features are interpreted cautiously, together with the patient's clinical history. An accurate diagnosis on FNA will prevent unnecessary surgery. Diagn. Cytopathol. 2013. © 2011 Wiley Periodicals, Inc.     

Alveolar rhabdomyosarcoma presenting as subacute intravascular coagulation.

A patient with rhabdomyosarcoma is described in whom the presenting clinical and laboratory features were those of disseminated intravascular coagulation. The patient's rapid downhill course was primarily expressed by haemorrhagic tendency. An alveolar rhabdomyosarcoma, affecting many organs, including vascular and cardiac lumina, was found at necropsy and was considered to be the cause of the consumption coagulopathy.     

大鼠迷走神经背核的细胞构筑及其P物质分布的免疫组化研究

用图像分析仪测量细胞参数、免疫组化技术结合显微分光光度计测定P物质样免疫反应颗粒的吸光率,对大鼠迷走神经背核的细胞构筑及其P物质的分布情况进行了探讨。 1.大鼠迷走神经背核细胞可分为小、中及较大三型,分别占所测细胞总数的50.83％、44.17％及5％。小细胞主要分布于核团吻侧部,中细胞散在于吻、中、尾各部,较大细胞仅位于中间部及尾侧部。各型细胞在核团内、中、外三份的分布无一定规律。 2.P物质样免疫反应细胞主要位于大鼠迷走神经背核的尾侧部,大多呈椭圆及圆形,可见1～2个突起,并多数分布于核团的内侧份。 3.大鼠迷走神经背核有丰富的P物质样免疫反应纤维,核团吻侧部及中间部纤维呈密集点状,尾侧部为短串珠状。 4.核团吻侧部及中间部P物质相对含量明显多于尾侧部。     

Alveolar rhabdomyosarcoma. Demonstration of the muscle type of intermediate filament protein, desmin, as a diagnostic aid.

Three cases of soft-tissue sarcomas with the characteristic histologic features of alveolar rhabdomyosarcoma, but lacking cytoplasmic cross-striations, were studied ultrastructurally and immunohistochemically to confirm the diagnosis and evaluate the histogenesis. The results showed that it was not possible to judge the skeletal muscle derivation of the cells at the ultrastructural level. However, immunohistochemically, the results of every case were positive for desmin-the muscle type of the intermediate filament protein. The results suggest that demonstration of desmin may be a helpful adjunct tool in the diagnosis of poorly differentiated alveolar rhabdomyosarcomas.     

Pleomorphic rhabdomyosarcoma in children: four cases in the pediatric age group

Pleomorphic rhabdomyosarcoma is considered rare and controversial, especially in children. Although pleomorphic rhabdomyosarcoma has been observed in children, its sparcity has taken it out of current childhood rhabdomyosarcoma classifications. We report four pediatric cases of pleomorphic rhabdomyosarcoma, review morphologic, immunohistochemical, and ultrastructural features, and discuss the rare need to include this category in children. The Soft Tissue Registry of the Armed Forces Institute of Pathology was searched for cases coded as "pleomorphic rhabdomyosarcoma" from 1970 to the present. Only cases in patients less than 21 years old were included. Clinical data, morphology, and immunohistochemical stains were reviewed and follow-up was obtained. Electron microscopy was performed on two cases. Molecular analysis by polymerase chain reaction was performed on one case with available material. Of four patients included, there were three boys and one girl. Patient ages ranged from 9 months to 10 years (median, 4.5 years). Tumors were located on the chest wall (n = 2) and one each on the upper and lower extremities. Tumor size ranged from 4.0 to 10.0 cm (median, 7 cm). Grossly, the tumors were lobulated and circumscribed. Microscopically, architectural patterns varied from solid to fascicular or storiform. All tumors had large, often multinucleated, polygonal, spindled or strap-like rhabdomyoblasts with abundant eosinophilic cytoplasm. Nuclear characteristics ranged from hyperchromatic to vesicular. Most tumor cells had large prominent nucleoli. Background rhabdomyoblasts varied from spindled to polygonal. No tumors displayed areas typical of embryonal or alveolar rhabdomyosarcoma. All tumors exhibited atypical mitotic figures. Immunohistochemistry revealed that the tumors were positive for the following markers: desmin (3/4), myoglobin (4/4), myoD1 (3/3), myf4 (3/3), and MSA (4/4). The two cases studied by electron microscopy both showed evidence for skeletal muscle differentiation. One case showed no evidence for a t(2;13) or t(1;13) translocation. Two patients were alive with no evidence of disease at 12 and 25 years. One patient was dead of disease at 9 years. Pleomorphic rhabdomyosarcoma is rare but exists in children. The diagnosis should be considered in pleomorphic sarcomas exhibiting skeletal muscle differentiation, which are otherwise devoid of typical areas or chromosomal changes of embryonal or alveolar rhabdomyosarcoma.     

右心房黏液瘤临床病理分析并文献复习

目的：探讨右心房黏液瘤的临床病理学特点及诊断要点。方法：对2例右心房黏液瘤进行临床资料、病理形态学及免疫组织化学观察,并结合文献对其诊断及鉴别诊断进行探讨。结果：镜下黏液瘤细胞呈单个散在、条索状或呈血管形成的指环样结构排列,黏液样基质在血管周围形成晕环;瘤细胞形成原始或分化的血管趋势,基质内淋巴细胞、浆细胞浸润;瘤细胞细长、梭形或星状,胞浆嗜酸性,胞核卵圆形、圆形或细长形;瘤细胞聚集呈球状,胞浆嗜酸性,胞核卵圆形或梭形。结论：右心房黏液瘤,非常少见,因此缺乏对其认识,从而易与其他肿瘤混淆导致误诊。提高对心房黏液瘤的认识,对避免误诊是至关重要的。     

Metastatic behavior of rhabdomyosarcoma

Embryonal and alveolar rhabdomyosarcoma probably have the same histogenesis, yet alveolar rhabdomyosarcoma has a considerably worse prognosis and is generally less responsive to treatment. The metastatic behavior of rhabdomyosarcoma in 22 autopsied subjects was analyzed with respect to the histologic subtypes of the tumors to determine whether the more aggressive behavior of alveolar rhabdomyosarcoma was related to greater tumor burden and/or more widely disseminated metastases. Subjects with embryonal rhabdomyosarcoma tended to survive longer and had greater numbers of metastatic sites at autopsy compared to subjects with alveolar rhabdomyosarcoma, but these differences were not statistically significant. In addition, there were no significant differences in the overall distribution of metastases between embryonal and alveolar rhabdomyosarcoma. The most frequent sites of metastases from both types of tumor were soft tissues (73%), serosal surfaces (64%), lymph nodes (64%), lungs (55%), and bone marrow (50%). The findings suggest that the metastatic behavior of rhabdomyosarcoma does not vary as a function of length of survival, mode of therapy, or histologic subtype. We propose that factors such as stage of disease at presentation and location of the primary tumor, rather than histologic subtype, are probably the most important variables governing the therapeutic responsiveness of rhabdomyosarcoma.     

Nodular fasciitis in the submandibular gland

Nodular fasciitis (NF) is a benign, proliferative lesion of myofibroblasts. The most common site of the lesion is in the upper extremities or trunk. NF in the head and neck is next in frequency and is the most common site in infants and children. In adults, NF in the submandibular region is very rare. We report a case of a 53-year-old man with a submandibular mass, rapidly growing for 10 days. Cytologic findings revealed a few sheets or clusters of small, uniform spindle cells. The uniform spindle cells had centrally located round to ovoid nucleus, but there were no nuclear atypia and atypical mitosis. There were focally loose stroma but we could not find chondroid or myxoid components. A partial parotidectomy was performed. We describe a case of NF in the submandibular region, adjacent to the parotid gland and review the clinical, cytologic, histologic, and immunohistochemical features of NF.     

硬化性横纹肌肉瘤的临床病理学分析

目的探讨硬化性横纹肌肉瘤（SRMS）的临床病理学特征,以及与胚胎性横纹肌肉瘤（ERMS）和腺泡状横纹肌肉瘤（ARMS）之间的关系。方法观察4例SRMS的临床特点、光镜形态,以免疫组织化学染色[En Vision法;波形蛋白、结蛋白、α-平滑肌肌动蛋白（α-SMA）、肌特异性肌动蛋白（MSA）、生肌蛋白、肌调节蛋白（MyoD1）、高分子量钙调结合蛋白（h-CALD）、CD31、CD34、第Ⅷ因子相关抗原、S-100蛋白、细胞角蛋白（AE1／AE3）和问变性大细胞淋巴瘤激酶（ALK1）]确定免疫学表型。结果4例均发生于成年人,平均年龄41．5岁。男性2例,女性2例。肿瘤分别位于左腕部、右大腿、右颊部和右面部,直径大小为2．5—10．0cm,平均5．7cm。镜下以含有大量玻璃样变的基质为特征,类似原始的骨样组织或软骨样基质。瘤细胞主要由原始的小圆形细胞组成,其排列方式呈多样化,包括条束状、索状、列兵样、梁状、微腺泡状和假血管样排列等。除1例可见少量的横纹肌母细胞外,其余3例均未见横纹肌母细胞,也未见花环状多核巨细胞。2例的局部区域还含有梭形细胞成分,其中1例类似梭形细胞横纹肌肉瘤,另1例类似周围神经肿瘤。免疫组织化学标记显示,瘤细胞弥漫强阳性表达MyoD1,而结蛋白多为灶性表达,生肌蛋白多为阴性或仅为灶性阳性。3例表达MSA,2例表达Ot-SMA,但不表达h-CALD。S-100蛋白、CD31和ALK1等标记均为阴性。结论SRMS在形态上和免疫学表型上与ERMS和ARMS均有所不同,但在细胞遗传学上与ERMS关系密切。熟悉SRMS的形态特征和免疫学表型有助于识别这种少见的横纹肌肉瘤亚型及与其他硬化性肿瘤相鉴别。