Malignant granular cell tumour with intraperitoneal dissemination

An unusual case of a malignant granular cell tumour involving the peritoneal cavity and probably arising in the pelvis is reported. The light microscopic diagnosis was confirmed by electron microscopy and immunohisto-chemistry.     

恶性颗粒细胞瘤临床病理、免疫组化和超微结构观察

目的：探讨恶性颗粒细胞瘤的病理学诊断和鉴别诊断要点及组织学起源。方法：对３例恶性颗粒细胞瘤进行临床病理、免疫组化及超微结构观察研究。结果：男性２例，女性１例，平均年龄为４９岁。部位分别为颈部１例，右大腿２例。其中２例分别于术后２年半及７年复发，并伴有区域淋巴结转移。组织学上３例与良性颗粒细胞瘤十分相似，局部区域出现梭形瘤细胞，空泡状核及明显的核仁，其中１例在肿瘤的周边部可见到瘤细胞与外周神经束支之间有直接移行关系。免疫酶标结果显示瘤细胞强阳性表达Ｓ－１００蛋白和神经特异性烯醇化酶（ＮＳＥ），１例电镜检测显示胞浆内充满膜包被复合性溶酶体。结论：对临床明显恶性而组织学上却极似良性的恶性颗粒细胞瘤，以下几点能提示恶性诊断：（１）肿瘤超过４ｃｍ；（２）核分裂象超过２个／１０ＨＰＦ；（３）核呈空泡状并有明显的核仁；（４）出现梭形瘤细胞；（５）有肿瘤性坏死。此外，免疫组化标记及超微结构观察有助于鉴别诊断及揭示组织学起源。     

Histogenesis of granular cell tumour--an immunohistochemical and ultrastructural study

Fifteen cases of granular cell tumour of superficial soft tissues or tongue were immunohistochemically evaluated for different types of intermediate filament proteins and for laminin, a glycoprotein of basal laminae. Four of the tumours were studied ultrastructurally. The tumour cells appeared to contain only vimentin-type of intermediate filament protein. The lobules of tumour cells were surrounded by laminin-positive material, but in contrast to schwannomas and neurofibromas, the individual tumour cells were not covered by laminin. In line with the immunohistochemical observations, by electron microscopy basal lamina-like material could not be demonstrated between individual cells, but only surrounding groups of cells. Lysozyme, a histiocytic marker, was absent in the tumour cells. Our results do not confirm any particular cell type for the histogenetic origin of granular cell tumour, but suggest that it may rather be derived from uncommitted possibly nerve-related mesenchymal cells.     

Dermal non-neural granular cell tumour (so-called primitive polypoid granular cell tumour): a distinctive entity further delineated in a clinicopathological study of 11 cases

AIMS: Cutaneous and soft tissue granular cell tumour is a well-characterized benign neoplasm of neural origin. However, there remains a subcategory of granular cell tumour, first described by Le Boit as 'primitive polypoid granular cell tumour', that shows no obvious line of differentiation. The aim of this study is to further the characterization of this lesion by undertaking a clinicopathological review. METHODS AND RESULTS: Eleven cases of dermal non-neural granular cell tumour were retrieved from one of the authors referral archives (E.C.) and both the histology and immunohistochemistry reviewed. Clinical data with follow-up were obtained from the referring pathologists. The lesions most commonly occurred in young to middle-aged adults (nine cases, median = 33 years, age range 6-56 years), with a slight female predominance. They presented as painless nodules, mainly on the extremities or face. Local excision was the treatment of choice and up to date follow-up reveals no sign of recurrence. Histologically, eight cases were polypoid, while three cases were endophytic. The tumours were composed of elongated spindle-shaped to polygonal or round cells with prominent granular cell change, and tumour nuclei showing mild focal atypia to rare moderate atypia. Mitotic activity ranged from one to nine mitoses per 10 high-power fields (median = 2, mean = 3.8). Immunohistochemical labelling of the tumour cells demonstrated expression for NKI-C3 (n = 11), focal, weak positivity for CD68 (n = 10) and FXIIIa (n = 2). There was negative staining for S100 protein, smooth muscle actin, Melan-A, CD34, desmin and cytokeratin. CONCLUSIONS: This analysis of 11 cases contributes to the characterization of this recently described entity, which despite some atypical histological features and no obvious line of differentiation, behaves in a completely indolent fashion.     

Malignant granular cell tumour (myoblastoma) of the vulva: report of a case and review of the literature

A case of malignant granular cell tumour (myoblastoma) which metastasized from the vulva to the regional lymph nodes is presented. Electron microscopy of the metastases demonstrated the presence of numerous intra-cytoplasmic lysosomes, a feature characteristic of these neoplasms, and the neoplasm was shown to stain strongly for carcinoembryonic antigen (CEA) using the immunoperoxidase technique. Previous reports in the literature of malignant granular cell tumours are reviewed and discussed.     

Malignant renal rhabdoid tumour. Immunohistochemical and ultrastructural studies

In an effort to establish their possible histogenesis, three cases of renal rhabdoid tumour and their metastases were studied both by a battery of polyclonal and monoclonal antibodies using the avidin-biotin-peroxidase complex technique and by electron microscopy. Vimentin was demonstrated in renal rhabdoid tumour in two cases and in all metastatic deposits. Cytokeratin (39, 43 and 50 kD) was not demonstrable in the three renal rhabdoid tumours, but was strongly positive in all metastatic lesions in one case. Epithelial membrane antigen was present in one renal rhabdoid tumour and in pulmonary metastases in two cases. Ultrastructural study showed epithelial differentiation in all tumours: basal lamina and convergent tight junctions were demonstrated; intracytoplasmic intermediate filaments were present in all primary and metastatic tumours. Rhabdoid tumours thus exhibited heterogeneous immunophenotypic expression suggesting that they are derived from mesenchymal cells which are capable of differentiating into epithelial cells.     

Malignant granular cell tumor. A clinico-pathologic and ultrastructural study of a case

The clinical and light- and electron-microscopic features of a malignant granular cell tumor in a 64-year-old man are reported. The ultrastructural appearance, with abundance of cytoplasmic "phagosomes", may help in the differential diagnosis. The light-microscopic findings and some ultrastructural observations support the suggestion that the tumor originated from peripheral nerves, and that a close relationship exists between malignant granular cell tumors and malignant Schwannoma.     

Pure Sertoli cell tumour of the ovary: report of a case with ultrastructural observations

A case of pure Sertoli cell tumour of the ovary is described. Unusual features were its great size, lack of clinical hormonal activity and the presence of granulomas in its interstitium. Mitochondria with tubular christae are described for the first time in such a tumour. These, together with lipid droplets and plentiful smooth endoplasmic reticulum, suggest a potential for steroid hormone synthesis which was not expressed clinically. Dense membrane-bound granules of undetermined type were present in large numbers. The presence of cilia and microvilli, and the absence of annulate lamellae and Charcot-Böttcher crystalloids, suggests that the tumour cells may not be truly of Sertoli type.     

Rhabdoid tumour of the bladder: a histological, ultrastructural and immunohistochemical study

A rapidly fatal bladder tumour which had the features of a rhabdoid tumour was studied by sequential biopsies and at autopsy. This is the first rhabdoid tumour recorded at this site and the first in which there was co-existent transitional cell carcinoma. The possibility that rhabdoid tumour is histogenetically heterogeneous is discussed.     

Malignant rhabdoid tumour of the uterus: an immunohistochemical and ultrastructural study

Summary Malignant rhabdoid tumours (MRTs) are highly aggressive neoplasms which most frequently occur in the kidney of young children. Several cases of primary MRT occurring in extra-renal sites have been reported, particularly in the soft tissues. We report a case of primary MRT of the uterus, a very rare site for this neoplasm, with morphological, immunohistochemical and ultrastructural features corresponding to restrictive morphological criteria for MRT. The possible differential diagnoses were considered.     

Ossifying fibromyxoid tumour of soft parts: immunohistochemical and ultrastructural analysis

Ossifying fibromyxoid tumour of soft parts is a recently described benign neoplasm which usually presents in soft tissue. The histogenesis or pattern of differentiation is a source of controversy. Thirteen cases are reported herein. All arose in adults, principally on the upper trunk or head and neck region. None recurred. All but one tumour showed a shell of lamellar bone, laid down by reactive osteoblasts, at the tumour-host interface. The characteristic laciform, glomoid and fascicular patterns, usually in combination, necessitate differential diagnosis from chondroid, smooth muscle and neural tumours especially. Immunohistochemistry revealed positivity for S-100 protein in 10/12, desmin in 7/10 and smooth muscle actin in 4/8. Ultrastructural examination of four cases revealed prominent intermediate filaments, without myofilamentous organization, and a discontinuous external lamina. Immunoelectronmicroscopy localized desmin positivity to the filamentous meshwork. Differentiation therefore appears to take the form of an incomplete neural and smooth muscle phenotype, without evidence of complete maturation.     

Adenomyoepithelioma of the skin: a case report with immunohistochemical and ultrastructural observations

 

Aims:

The histological, immunohistochemical and electron microscopic features of a primary adenomyoepithelioma of skin, a rare sweat gland tumour, are reported.  

Methods and results:

The tumour occurred on the back of a 92-year-old woman. It was composed of well-formed tubules lined by epithelial cells surrounded by clear or spindled myoepithelial cells. Immunohistochemically, the epithelial cells exhibited strong cytokeratin (CAM5.2) and weak carcinoembryonic antigen positivity. The myoepithelial cells showed diffuse positivity for smooth muscle actin and focal positivity for S100 protein. Ultrastructurally, the myoepithelial cells contained myofilaments with focal densities and hemi-desmosomes. They were limited by well-formed basal lamina. The tumour was associated with a small eccrine spiradenoma.  

Conclusion:

We predict that the tumour will behave in a benign fashion. There is no evidence of recurrence or metastasis 28 months later.     

Malignant haemangiopericytoma of lung: a cell culture and ultrastructural study

Malignant haemangiopericytoma was grown in vitro to assess the histogenesis of the tumour. Disaggregated tumour cells maintained in serum-supplemented Waymouth's MB 752/1 medium showed monolayers of homogeneous spindle cells. Post-confluent cultures exhibited hillocks or plaques of multilayer growth within which considerable amount of basal lamina-like material and fibrillar matrix were present between cells. Delicate basal laminae were also expressed on cell surfaces facing matrix material. Other consistent features were attenuated cytoplasmic processes, desmosome-like junctions, abnormal mitochondria and a paucity of intracellular filaments. The three-dimensional organization of tumour cells with concomitant expression of differentiated phenotype in vitro has significant implications on the cell of origin and differentiation process of haemangiopericytoma.     

Malignant rhabdoid tumour of the bladder with immunohistochemical and ultrastructural evidence suggesting histiocytic origin

A malignant rhabdoid tumour of the bladder is reported from a girl aged 6. Detailed immunohistochemical and ultrastructural investigations provide evidence which suggests a histiocytic origin for this controversial neoplasm.     

Perivascular myoma: case report with immunohistochemical and ultrastructural studies

A subcutaneous myopericytoma-type perivascular myoma arising in the elbow of a 61-year-old woman is described. The tumor was well demarcated and consisted microscopically of small ovoid and spindle cells arranged in a concentric fashion, surrounding small to medium-sized vessels and imparting a superficial resemblance to hemangiopericytoma. In some areas, the cellular whorls were separated by myxoid stroma. Cells located between cellular whorls appeared immature with scant cytoplasm but did not show distinct nuclear anaplasia, increased mitoses or foci of necrosis. Immunohistochemistry showed that constituent cells were positive for alpha-smooth muscle actin and desmin. Electron microscopy disclosed that the immature-looking cells, as well as the ovoid and spindle-shaped cells, possessed focal densities along with thin filaments, subplasmalemmal densities, pinocytotic vesicles and an external lamina. These ultrastructural and immunohistochemical features indicate a myoid nature of pericytic cells and justify this type of neoplasm being categorized as perivascular myoid tumor.     

Gynandroblastoma of the ovary: A case report with an immunohistochemical and ultrastructural study

An ovarian gynandroblastoma in a 60-year-old woman is described. The cut-surface of the right ovary showed multiple macrofollicles separated by white fibrous tissues and multiple ill-defined yellowish nodules. The tumour consisted of substantial amount of a granulosa cell element and a Sertoli cell element with intermingled Leydig cells. Immunohistochemically, the tumour cells in both the granulosa cell and Sertoli cell elements were positive for cytokeratin CAM5.2. The granulòsa cell element showed strong membrane staining of Ewing's sarcoma antigen 013 and the Sertoli cell element was focally positive. Vimentin was observed in both the Sertoli cell element and the granulosa cells. Both elements and the Leydig cells were uniformly negative for epithelial membrane antigen, muscle specific actin, CD31 and CD34. The tumour was aneuploid by flow cytometry. The patient was well with no evidence of tumour five months after surgery.     

Elastosis in granular cell tumours: prevalence and distribution

Elastosis, a previously unreported feature, was found to be common in granular cell tumours. Twenty-four of 28 consecutive granular cell tumours were shown to display elastosis. The elastosis was found in three main sites: adventitial, interstitial and septal. Elastosis is statistically associated with the fibrosis present within this tumour. It appears that elastic fibres are synthesized by connective tissue cells, possibly induced by the tumour cells.     

Malignant ovarian mixed germ cell tumour: a rare combination

Ovarian germ cell tumours are very rare and affect mainly young girls and women. Due to this, the conservation of reproductive potential is of great concern. One of the most remarkable advances in oncology is in the treatment of malignant ovarian germ cell tumours. Two histological groups are distinguished: dygerminomas, equivalent to testicular seminomas, and non-dysgerminomatous tumours. We report a case of a 30-year-old nulliparous woman who presented with persistent per vaginal bleeding and was found to have a malignant mixed germ cell tumour comprising of both embryonal carcinoma and choriocarcinoma.     

Malignant Warthin''s tumour: a case study

Malignant transformation of a benign Warthin's tumour (adenolymphoma) is extremely rare. The light microscopic and ultrastructural features of an adenocarcinoma arising in a Warthin's tumour in the parotid gland are described. Light microscopy demonstrated a transition zone from the benign to the malignant component, and the malignant cells revealed oncocytic features by both light- and electronmicroscopy.