Cerebellar Liponeurocytoma, an Unusual Tumor of the Central Nervous System--Ultrastructural Examination.

Cerebellar liponeurocytoma is a rare tumor of the central nervous system which shows neuronal and variable astrocytic differentiation, along with foci of lipomatous differentiation. It is usually located in the cerebellum, and may be mistaken for medulloblastoma with lipidized cells or lipomatous ependymoma. Histopathological examination, supplemented by immunohistochemistry and electron microscopy, is required to distinguish between these entities. This 35-year-old male presented with vomiting and headache for three months, followed by gait imbalance. Neurological examination showed positive cerebellar signs with ataxic gait. Magnetic resonance imaging showed a lesion measuring 4.4 cm× 4.3 cm× 3.9 cm involving the cerebellum. The patient underwent midline suboccipital craniotomy to excise the tumor. Histopathological examination showed a circumscribed, cellular tumor composed of round to polygonal cells with moderate cytoplasm and minimal pleomorphism. Clear intracytoplasmic vacuoles were seen within the tumor cells. These tumor cells were immunopositive for synaptophysin, NSE, and MAP-2, confirming their neurocytic origin. On ultrastructural examination, lipid vacuoles as well as dense-core neurosecretory granules were identified within these neurocytic cells, confirming the diagnosis of liponeurocytoma. No cilia, microvilli, or gap junctions were identified in the tumor cells, ruling out the possibility of lipomatous ependymoma. The differentiation of liponeurocytoma from its morphological mimics is imperative, as their treatment differs drastically. The role of electron microscopy is extremely important in this differential diagnosis.

A case of pure uterine lipoma: immunohistochemical and ultrastructural focus.

BACKGROUND: Pure uterine lipoma is a rare clinical event and only a few cases have been reported in literature. The histogenesis of these lesions is still debatable. Preoperative diagnosis is difficult and should be pathologically confirmed postoperatively. CASE: We report the case of a 58-year-old woman who presented with pelvic pain and postmenopausal uterine bleeding. The hysterectomy specimen showed a pure intramural lipoma of the uterus. An immunohistochemical study revealed that the lipomatous tissue was reactive to S-100, vimentin, actin and desmin. Electron microscopy examination revealed bundles of spindle cells with intracytoplasmatic vacuoles and parallel-arranged intermediate filaments in the surrounding zone, in which adipose cells were mixed with muscular cells. DISCUSSION: Clinical and histological diagnosis of pure uterine lipomas are described and a possible involvement of fatty metaplasia of smooth muscle cells in the development of pure uterine lipomas is discussed.

Mediastinal thymolipoma: a rare occurrence with striated myoid cells.

We describe a 49-year-old man who presented to hospital with unstable angina and who underwent emergency coronary artery bypass grafting. During the surgical procedure, an incidental anterior mediastinal mass was discovered. Histological examination of this mediastinal mass revealed a thymolipoma containing numerous polygonal, striated myoid cells that were immunoreactive for desmin, muscle-specific actin and myoglobin. Electron microscopy demonstrated numerous Z band structures confirming myoid differentiation. Even though the appearance of myoid cells in thymolipoma may be alarming, this tumor should be recognized as a benign entity. Thymolipomas containing striated myoid cells should be differentiated from more ominous thymic neoplasms, including teratomas and thymic liposarcomas. To our knowledge, this is only the third reported case of thymolipoma containing striated myoid cells.

Pulmonary lipomatous hemangiopericytoma: report of a rare tumor and comparison with solitary fibrous tumor.

Lipomatous hemangiopericytoma is a rare mesenchymal tumor showing areas of lipid-containing cells admixed with a spindle-cell component. Like other hemangiopericytomas, it shows a similar vascular pattern to solitary fibrous tumor and, partly for this reason, it and other hemangiopericytomas have been subsumed into solitary fibrous tumor. The present study provides a comprehensive documentation of a single case of pulmonary lipomatous hemangiopericytoma of the lung, the first to be described at this site, and compares it with solitary fibrous tumor, in terms of clinical, histological, immunohistochemical, ultrastructural, and cytogenetic findings. Apart from the lipid-laden-cell component, pulmonary lipomatous hemangiopericytoma and solitary fibrous tumor were similar histologically. Bcl-2 was positive in both. CD34 was minimally expressed in pulmonary lipomatous hemangiopericytoma, which possessed some non-descriptive intercellular junctions, a feature shared by solitary fibrous tumor, which was CD34 positive. However, one of the latter was rich in gap junctions, a feature consistent with strong connexin (Cx) 43 staining and the existence, hitherto unappreciated, of a CD34/Cx43-positive tumor cell network. In pulmonary lipomatous hemangiopericytoma, chromosomal deletions of 43-44, X, -Y were found. In solitary fibrous tumor, 46, XY, del(13)(q?) abnormalities and abnormalities involving chromosome 10 were frequently observed. These similarities and differences are discussed in the context of the currently favored diagnostic fusion of hemangiopericytoma and solitary fibrous tumor.

Angiomatous spindle cell lipoma: Report of three cases with immunohistochemical and ultrastructural study and reappraisal of former 'pseudoangiomatous' variant.

Pseudoangiomatous spindle cell lipoma (PASCL) is a rare variant of spindle cell lipoma that is composed of spindle fibroblast-like cells and mature adipose cells, and that is further characterized by intratumoral branching spaces regarded originally to be non-vascular (hence the adjective 'pseudoangiomatous'). Reported herein are three cases of this tumor, with literature review of all six cases described previously. PASCL appears to have clinical features (age, sex, location, prognosis) similar to those of conventional spindle cell lipoma (SCL). Immunohistochemically, the spindle cells in all of the present cases were positive for CD34, as in conventional SCL. All lesions had at least mild positivity for sex steroid hormone receptors. The cells lining intratumoral dilated and branching spaces typical of this variant were strongly positive for lymphatic endothelial marker D2-40 in all cases, and for vascular markers CD31 and F-VIII in one case. Ultrastructural study confirmed the endothelial nature of these cells. The results indicate that pseudoangiomatous SCL is truly angiomatous and should be termed as such. It could represent a sex steroid hormone-dependent lesion.

Light and electron microscopic findings in a patient with orbital myolipoma.

BACKGROUND: This report describes the pathology of myolipoma, which is a benign soft tissue tumor and is a very rare tumor of the orbit. METHODS: A 41-year-old woman complained of exopthalmos of her right eye. By MRI (magnetic resonance imaging) examination, the tumor (30x20 mm) was located in the extraconus of four recti muscles. A part of the tumor was excised and examined with both light and electron microscopes and immunohistochemically. RESULTS: Light microscopic inspection revealed the tumor was formed by spindle-shaped cells, which were adipocytes and smooth muscle. Electron microscopy showed the cytoplasm contained parallel filaments associated with fusiform densities. Immunohistochemical staining showed smooth muscle stained with SMA (smooth muscle actin). Angiomyolipoma was excluded by negative staining for HMB45, Melan A. CONCLUSION: To our knowledge, only one case of orbital myolipoma has been reported. The intimate relationship between the two cell types led us to conclude that the tumor was dimorphic and could be classified correctly as a myolipoma.

Cerebellar central liponeurocytoma.

Cerebellar liponeurocytoma is a rare, benign neuroepithelial tumor that occurs exclusively in the cerebellum of adults. Its salient histological features include advanced neuronal/neurocytic differentiation, focal vacuolated cells resembling mature adipose cells, low mitotic activity, and lack of endothelial proliferation and/or necrosis. The morphological appearance of this neoplasm can be confused with that of oligodendroglioma, neurocytoma, ependymoma, medulloblastoma, hemangioblastoma, metastatic renal cell carcinoma, and other clear cell carcinomas. Its full biological potential and histological features, however, have not been fully exploited due to the rarity of this tumor. The authors describe a case with clinical, imaging, histological, immunohistochemical, and ultrastructural features.

Hibernoma: a report of 2 unusual cases with a review of the literature.

Hibernomas are rare neoplasms composed of brown adipose tissue. The behavior of these neoplasms has been described as uniformly benign in humans. The only recurrence cited in the English literature involved a sarcoma with hibernoma-like features, which was reported in abstract form. We present 2 cases of hibernoma, one that continued to grow following partial excision and another at an unusual site (anterior abdominal wall). Both of these tumors overexpressed p53 protein by immunohistochemistry, which was a novel finding. A review of the literature highlights recent advances that may help confirm the diagnosis and explain the biology of these rare tumors.

Cerebellar liponeurocytoma: immunohistochemical and ultrastructural study of a case.

The clinical, histopathological, and ultrastructural features of a cerebellar liponeurocytoma are reported. The tumor, a 3-cm mass localized in the right cerebellar hemisphere, was resected from a 61-year-old man clinically presenting with symptoms of intracranial hypertension. The lesion was composed of small, in some areas closely packed, medulloblastoma-like cells with prominent areas of lipidization phenomena. Moreover, mitoses, cellular atypia, and numerous vascular structures were focally observed. Glial and neuronal differentiation was immunohistochemically noted (glial fibrillary acidic protein, synaptophysin, neurofilaments, and neuron-specific enolase positivity). The p53 oncoprotein was detected in the majority of neoplastic cells and a moderate proliferation activity, evaluated by Mib-1 antibody, was focally appreciated. Ultrastructural study did not show evident neuritic processes, synapses, or dense core neuroendocrine granules. This cerebellar tumor previously called lipidized medulloblastoma and recently renamed cerebellar liponeurocytoma is considered an adult neoplasm with excellent prognosis histologically presenting prominent lipidized areas and, at the immunohistochemical and ultrastructural level, both glial and neuronal differentiation. The present study provides the first description of a less differentiated and histologically more aggressive form of this unusual tumor.

Spindle-cell lipoma of the cheek: a case report.

Spindle-cell lipoma (SCL) is a distinct histological variant of lipoma. Clinically, it appears as a solitary, subcutaneous, circumscribed lesion. SCL accounts for about 1.5% of all adipocytic tumours. Only nine cases of intraoral SCL were found in the literature. Microscopically, mature adipocytes and spindle cells are immersed in a myxoid stroma. SCL needs only local excision, and it does not recur.

The mineralocorticoid receptor mediates aldosterone-induced differentiation of T37i cells into brown adipocytes.

By use of targeted oncogenesis, a brown adipocyte cell line was derived from a hibernoma of a transgenic mouse carrying the proximal promoter of the human mineralocorticoid receptor (MR) linked to the SV40 large T antigen. T37i cells remain capable of differentiating into brown adipocytes upon insulin and triiodothyronine treatment as judged by their ability to express uncoupling protein 1 and maintain MR expression. Aldosterone treatment of undifferentiated cells induced accumulation of intracytoplasmic lipid droplets and mitochondria. This effect was accompanied by a significant and dose-dependent increase in intracellular triglyceride content (half-maximally effective dose 10(-9) M) and involved MR, because it was unaffected by RU-38486 treatment but was totally abolished in the presence of aldosterone antagonists (spironolactone, RU-26752). The expression of early adipogenic gene markers, such as lipoprotein lipase, peroxisome proliferator-activated receptor-gamma, and adipocyte-specific fatty acid binding protein 2, was enhanced by aldosterone, confirming activation of the differentiation process. We demonstrate that, in the T37i cell line, aldosterone participates in the very early induction of brown adipocyte differentiation. Our findings may have a broader biological significance and suggest that MR is not only implicated in maintaining electrolyte homeostasis but could also play a role in metabolism and energy balance.

[Lipomatous meningioma: report of two cases and review of the literature]. / Lipomatosus meningeoma: két eset ismertetése és irodalmi áttekintés.

Lipomatous meningioma is a benign tumor characterized either by an admixture of mature adipocytes and meningioma or the production of triglycerides by neoplastic meningothelial cells assuming a lipoblast-like appearance. The authors report on two instances of this exceedingly rare lesion occurring in the left middle cranial fossa and over the right frontal convexity of two female patients aged 79 years and 60 years, respectively. In the former, the tumor was an incidental autopsy finding, while the latter underwent surgery for symptoms of intracranial space occupation. Light microscopy showed interwoven islands of fatty tissue and transitional meningioma in the first case; whereas a monomorphous signet-ring cell phenotype prevailed in the second. Oil-Red-O staining confirmed the presence of neutral fat in both specimens. Immunohistochemical coexpression of epithelial membrane antigen, vimentin, and S100 protein supported the meningothelial origin of tumor cells. On the other hand, the CD 68 macrophage antigen was not detected. Cytoplasmic lipid droplets along with hallmarks of meningothelial differentiation were visualized ultrastructurally in part of the meningioma component of the first case and throughout the second. These findings are consistent with a metaplastic origin of the adipocytic element. Whatever its histogenesis, lipomatous meningioma may, on occasion, represent a major challenge with therapeutic implications for both preoperative imaging and histological diagnosis.

Dendritic fibromyxolipoma: clinicopathologic study of a distinctive benign soft tissue lesion that may be mistaken for a sarcoma.

Twelve cases are described of a distinctive benign soft tissue lesion that may be mistaken for a sarcoma. The tumors occurred in 11 men and a woman aged 33 to 81 years (mean, 64 years), and measured from 2 to 11 cm in greatest diameter (mean, 6 cm). They were grossly described as soft, well-circumscribed, yellow-gray, with a mucoid cut surface. All cases were superficially located in the subcutis or muscular fascia of the head and neck region or the chest and back. Histologically, the tumors were characterized by a proliferation of spindle or stellate fibroblastic cells variably admixed with mature adipose tissue embedded in an abundant myxoid and collagenized stroma. The spindle and stellate fibroblastic cells were characterized by slender dendritic prolongations of their cytoplasm, which appeared to extend for short distances along connective tissue planes. Electron microscopy in two cases confirmed the dendritic nature of the fibroblastic cells, which showed elongated cytoplasmic processes lacking external lamina and displaying foci of pinocytotic activity. Immunohistochemical studies in 11 cases showed strong positivity of the spindle cells with vimentin, CD34 and bcl-2, and negative staining for smooth muscle actin, muscle-specific actin (HHF35), desmin, S-100 protein, keratin, and EMA. Because of their prominent myxoid stroma and relatively large size, some of these tumors were initially misinterpreted as low-grade sarcomas. Clinical follow-up in five cases, however, showed that the patients were alive and well without evidence of recurrence between 5 and 13 years (mean follow-up, 8 years) after simple local excision. The present cases appear to represent a distinctive form of benign soft tissue neoplasm that should be distinguished from myxoid liposarcoma and other benign and malignant myxoid tumors of superficial soft tissues.

Benign spindle cell tumor of the breast with prominent adipocytic component.

We report a rare case of "benign spindle cell tumor of the breast with prominent adipocytic component" and present a review of the literature on the topic. The close morphologic resemblance with myofibroblastoma and spindle cell lipoma of the breast is emphasized and used as a starting point for diagnostic and histogenetic considerations. Although immunocytochemistry and electron microscopy showed features favoring a diagnosis of spindle cell lipoma, they were not conclusive. The tumor described here contributes to widen the spectrum of the benign spindle cell tumors of the breast variously named "benign spindle cell tumor," myofibroblastoma, spindle cell lipoma, and fibroma. It is important to distinguish this lesion from spindle cell metaplastic carcinoma and aggressive fibromatosis.

Myolipoma of the eyelid.

BACKGROUND: This report describes the pathology of a myolipoma which occurred in the eyelid. Myolipoma is a benign hamartomatous tumour in which smooth muscle cells are interspersed with adipocytes. PATIENT DETAILS: An irregular yellowish tumour (30 x 25 mm) with illdefined borders had been present for 50 years in the medial part of the left lower eyelid of a 67-year-old woman. The tumour was excised and studied by conventional histology, immunohistochemistry and transmission electron microscopy. RESULTS: The tumour was formed by bundles of spindle-shaped cells with cigarshaped nuclei intermingled with multiloculated clear cells containing small eccentric nuclei. By immunohistochemistry, positive staining of the spindle cells was restricted to smooth muscle actin and desmin; the clear cells were non-reactive with the immunohistochemical panel, but fat was identified within the cytoplasm. The ultrastructural features of the spindle cells were those of a leiomyoma, while the clear cells were classified as adipocytes. CONCLUSION: This tumour was considered to originate from the media of blood vessels within the tumour.

Synovial metaplasia in lipoma.

We present a unique case of a lipoma with widespread synovial metaplasia. A 52-year-old woman had a recurrence 1 year after excision of a subcutaneous lipoma of the neck. Histologically, the primary tumor was an ordinary lipoma. The recurrent tumor was a myxoid lipoma with synovial metaplasia. The synovial metaplastic process manifested as labyrinthlike clefts, which were lined by one or more synovial-like cell layers. Ultrastructurally, the synovial metaplastic cell had secretory, phagocytic, and fibroblastlike features.