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PubMedDate not supplied

Pleomorphic xanthoastrocytoma, a distinctive astroglial tumor: neuroradiologic and pathologic features.

PURPOSE: To analyze and discuss CT and MR features of pleomorphic xanthoastrocytoma (PXA) and present salient histopathologic features of this distinctive astroglial tumor. METHOD: CT, MR, and histopathologic studies on seven patients with the histologic diagnosis of PXA were reviewed retrospectively. RESULTS: All patients were in their first 3 decades of life when first diagnosed and demonstrated peripherally situated supratentorial tumors of varying size involving the superficial cortex and leptomeninges. Five of six cases examined with CT showed areas of mixed attenuation with four demonstrating well-demarcated enhancement. MR demonstrated low or mixed signal intensity on T1-weighted and high or mixed signal intensity on T2-weighted sequences. All five who received gadopentetate dimeglumine showed well-defined enhancement. Three showed cyst formation. Typical histologic features included marked cellular pleomorphism with giant cells, bizarre nuclei, variable cytoplasmic lipidization and positive immunoreactivity for glial fibrillary acidic protein. Necrosis and endothelial-pericytic cell proliferation were absent. CONCLUSION: PXA has a highly suggestive neuroradiologic and distinctive histopathologic appearance.

Adolescent↗

PubMed1994

Malignant fibrous histiocytoma phenotype in pleomorphic sarcoma differentiation in recurrent disease.

Two cases of pleomorphic sarcoma with malignant fibrous histiocytoma phenotype that recurred following therapy with a distinctly different, more mature histologic appearance and immunohistochemical profile are reported. The first case recurred predominantly as extraosseous osteosarcoma at the same site 17 months after wide local excision. The second case recurred as widespread pulmonary, mediastinal, and hepatic metastatic disease 5 years after wide local excision and adjuvant local radiotherapy and chemotherapy with doxorubicin hydrochloride (Adriamycin) and cyclophosphamide (Cytoxan). Fine-needle aspiration of the pulmonary metastatic lesions showed predominantly spindle cells without any large, pleomorphic giant cells typical of malignant fibrous histiocytoma. The patient was treated by radiotherapy to the lung and mediastinum and by chemotherapy with ifosfamide and mesna. Biopsy of a metastatic scrotal skin nodule 9 months later showed a malignant spindle cell lesion with the histologic appearance and immunohistochemical phenotype of leiomyosarcoma. Retrospective immunohistochemical evaluation of the primary tumor showed focal desmin expression, suggesting focal leiomyosarcomatous differentiation. However, the large proportion of the primary tumor had the phenotype of malignant fibrous histiocytoma. These two cases illustrate an unusual finding of "differentiation" rather than "dedifferentiation" in a recurrent sarcoma. The transformation to osteosarcoma and differentiated leiomyosarcoma demonstrates the potential for phenotypic changes in soft-tissue sarcomas and suggests that the malignant fibrous histiocytoma phenotype and more-differentiated sarcomas such as extraosseous osteosarcoma or leiomyosarcoma are related in a common pathway in differentiation from a primitive mesenchymal stem cell.

Aged↗

PubMedDate not supplied

Intrathoracic pleomorphic liposarcoma in a child: study of an unusual case.

Primary intrathoracic liposarcoma is an extremely rare neoplasm in the pediatric age group. We report an unusual case of a 3-year-old boy who presented with a large intrathoracic tumor arising from the right chest wall. Biopsy showed a pleomorphic sarcoma with histological, immunohistochemical, and ultrastructural features of pleomorphic liposarcoma. The tumor displayed an aggressive clinical behavior, and the patient continued to have recurrences despite rigorous chemotherapy and radiation treatment. He subsequently died 2 years after the initial diagnosis.

Child, Preschool↗

PubMed1996

Pleomorphic xanthoastrocytoma.

Pleomorphic xanthoastrocytoma is an unusual brain tumour of children with a favourable prognosis. The tumour takes origin from subpial astrocytes of the cerebral cortex. Histologically proliferation of spindle cells, mono and multinucleated giant cells containing lipid droplets and the absence of necrosis are distinctive features. Herein we report the morphological and immunophenotypical features of a case of pleomorphic xanthoastrocytoma.

Astrocytoma↗

PubMed1997

[Pleomorphic xanthoastrocytoma].

Pleomorphic xantho-astrocytoma (PXA) is a relatively rare brain tumor of adolescents and young adults characterized by its superficial location with frequent involvement of the meninges, and its slow growth despite features of histological atypia. The authors present a retrospective immunohistochemical analysis of 8 surgically treated cases in order to determine the expression of glial and neuronal markers, and to assess the proliferating cell fraction. The study population comprised 1 female and 7 male patients with a mean age of 26.7 years, most tumors being located in one of the temporal lobes. Epilepsy predominated as a presenting symptom. Five cases were assigned WHO graded II, while the diagnosis of anaplasia (WHO grade III) was established in three, based either on elevated mitotic counts or the presence of necrosis. Immunostaining with the proliferation marker MIB-1 was present in 2.05% of cells in the former groups, while 4.66% showed labeling in the latter. Postoperative follow-up averaged 6.7 years, with only one recurrence of an anaplastic tumor. All tumors expressed some amount of glial fibrillary acidic protein and were shown to elaborate a characteristic pericellular reticulin network. There was focal reactivity for alpha-1-antitrypsin, but neither the monocyte-macrophage associated antigen CD68 nor lysozym could be detected in neoplastic cells. In 7 cases, scattered individual tumor cells exhibited synaptophysin positivity. The authors review problems and prognostic issues of the histologic diagnosis of anaplasia occurring in some 20% of the cases. A possible dysontogenic origin of PXA and its nosologic relationship to the so-called desmoplastic neuroepithelial tumors of infancy are discussed. This is the first study of pleomorphic xanthoastrocytoma in the Hungarian literature.

Adolescent↗

PubMedDate not supplied

A case of pleomorphic lipoma of the hypopharynx.

We report the first case of pleomorphic lipoma in the hypopharynx. A 69-year-old man was admitted because of a 12 year history of dysphagia and cough. Direct laryngo-pharyngoscopy revealed a polypoid, yellow, smooth, submucosal tumor with well defined borders. At CT scan the tumor showed a hypodense pattern and measured 3 cm. Microscopically the neoplasm was composed mainly by typical lipomatous tissue, bundles of collagen and pleomorphic multinucleated cells, some with floret-like appearance. Lipoblasts and mitoses were not found. Six months after resection there was no evidence of recurrence.

Aged↗

PubMed2026

Long-Term Survey to Assess Recurrence and Complications of Surgically Treated Pleomorphic Adenoma.

OBJECTIVE(S): To (1) report on the long-term recurrence rate of surgically managed pleomorphic adenoma (PA) and (2) assess long-term surgical outcomes after parotidectomyMethods:Cross-sectional survey of all patients with a pathologic diagnosis of PA who underwent surgical management in a tertiary care center from 01/1997 to 12/2023 and had access to an electronic patient portal. Surveys were delivered via electronic patient portal or phone call with 6 questions to assess recurrence and long-term surgical outcomes. Time to first recurrence and recurrence free survival were analyzed using a Kaplan-Meier curve. RESULTS: Seven hundred-forty-five patients met inclusion criteria and received the survey, with a 56% response rate (n = 420). The clinical median follow-up length was 51 days, which extended to a median length of 6.2 years following the survey. The median change in follow-up length was 5.37 years (P < .001). The recurrence rate was 1% at 5 years, with a median time to first recurrence of 7.54 years. 13% and 9% of responders reported facial asymmetry and incisional cosmetic concerns, respectively. 33% of patients reported experiencing Frey syndrome, which increased from 5.2% of survey responders with documented Frey syndrome at their original post-operative assessment. CONCLUSION: Long-term assessment of PA outcomes confirms low recurrence rates, suggesting regular surveillance of recurrence is likely unnecessary. Complaints of Frey syndrome increased in frequency with longer term follow-up, which should be considered during patient counseling. LEVEL OF EVIDENCE: 3.

Humans↗

PubMed2002

Pleomorphic adenoma of the submandibular gland: clinicopathological and immunohistochemical features of 60 cases in Brazil.

The submandibular gland is involved in only 5% to 10% of the salivary gland tumors, and pleomorphic adenoma (PA) is the most common tumor affecting it. This study describes the clinicopathological features and immunohistochemical expression of Ki-67 and p53 in 60 cases of submandibular salivary gland PAs. Most of the patients were in the third and fifth decades of life and 37 (62%) of them were women. Tumor sizes varied from 1 to 10 cm and the mean time between symptom onset and treatment was 52 months. Only 1 patient experienced local recurrence, 3 years after treatment. Histologically, most tumors consisted chiefly in a chondromyxoid stroma. Stroma-rich PAs were larger than stroma-poor ones (P<.02). All PAs were found negative for Ki-67 and p53. These results show that PAs of the submandibular gland are histologically similar to PAs of other salivary glands, and that they have a low proliferative rate and a good prognosis.

Adenoma, Pleomorphic↗

PubMed1988

Pleomorphic adenoma of the middle ear and mastoid with posterior fossa extension.

We describe the case of a pleomorphic adenoma of the middle ear and mastoid, with extension into the posterior cranial fossa. The lesion was characterized by extensive bony destruction and close proximity to the facial nerve. Ectopic or choristomatous salivary gland tissue within the middle ear cleft is the most probable origin of this tumor.

Adenoma, Pleomorphic↗

PubMed1996

Cytomorphology of a mixed pigmented/nonpigmented pleomorphic adenoma of the ciliary body: a diagnostic pitfall in the evaluation of intraocular tumors.

Intraoperative cytology is an alternative and sometimes preferred method to frozen section in the rapid evaluation of surgical biopsies. This report describes the cytological, histological, and immunohistochemical features of a case of mixed pigmented/nonpigmented pleomorphic adenoma of the ciliary body with clinical and morphological features reminiscent of malignant melanoma. The characteristic cyto-histomorphology of this unusual tumor and its distinguishing features from malignant melanoma are discussed.

Adenoma, Pleomorphic↗

PubMed1975

Pleomorphic adenoma of the salivary gland. With special reference to histochemical and electron microscopic studies and biochemical analysis of glycosaminoglycans in vivo and in vitro.

Pleomorphic adenoma of the salivary gland is classified into two types based on the morphology of the cells and biochemical analysis of glycosaminoglycans. Type 1: the tumors consisted mainly of spindle cells, producing a large amount of mucinous material, and contained not only chondroitin 4- and 6-sulfate and hyaluronic acid but also heparan sulfate. When the tumors of this type were dissociated and cultivated, spindle cells proliferated vigorously as mulitlayer and secreted mucinous substance into the intercellular space. The cells looked like non-epithelial cells in a conventional culture bottle, but showed an epithelial-like arrangement when they were cultivated in sponge matrix or inoculated subcutaneously into mice. Fine fibres stained with orceine or resorcin-fuchsin were observed in the intercellular material both in resected tissue and in culture. Type 2: the tumors consisted mainly of ductal cells, having no mucinous area, and contained little heparan sulfate. When the tumors were cultivated, the growth of epithelial-like polygonal cells was observed in monotonous pattern, producing scanty intercellular substance, and proliferating, as monolayer, much less rapidly than the cells of Type 1.

Adenoma, Pleomorphic↗

PubMed1979

Simple enucleation and radiotherapy in the treatment of the pleomorphic salivary adenoma of the parotid gland.

A series of 76 patients with a primary pleomorphic salivary adenoma treated by simple extracapsular enucleation and high energy radiation between 1963 and 1976 is presented. Analysis of the results has shown a very low incidence of complications with only minimal cosmetic deformity. Of the 76 patients, only 5 have been lost to follow-up, 3 patients have died of causes unrelated to the tumour and only 1 patient has developed a recurrence. Further local excision in this patient showed the histological appearances of adenocarcinoma.

Adenoma, Pleomorphic↗

PubMed1988

Management of pleomorphic adenoma of the parotid.

A series of 336 parotidectomies, carried out for benign disease has been followed for up to 20 years. One hundred and thirty primary pleomorphic adenomas were treated by wide excision of the tumour after identification of the facial nerve. Recurrence occurred in only one patient and, although 20 patients (15 per cent) experienced some degree of facial nerve weakness postoperatively, there was only one case of permanent weakness.

Adenoma, Pleomorphic↗

PubMed1989

Capsular penetration and perforation in pleomorphic adenoma of the parotid salivary gland.

Both penetration and perforation of the capsule were seen on the superficial aspect of pleomorphic adenomas. Only penetration was noted on the deep aspect, where the tumour was adjacent to the facial nerve. In terms of local recurrence this may explain why it appears to be safe to separate the deep aspect of the tumour from the facial nerve without a wide margin of normal parotid tissue.

Adenoma, Pleomorphic↗

PubMed1996

Clinical significance of the tumour capsule in the treatment of parotid pleomorphic adenomas.

The propensity of pleomorphic adenomas to recur is generally attributed to the biological nature of the tumour, and surgery close to the capsule is perceived as undesirable. At the Christie Hospital, Manchester, between 1947 and 1992, 475 tumours arising within the superficial portion of the parotid gland were treated by two surgical techniques: extracapsular dissection (380 patients) and superficial parotidectomy (95). Recurrence rates were 2 per cent in each group (median follow-up 12.5 years). Contact of the tumour with the facial nerve was recorded in 51 per cent of patients. There was no difference between the treatment groups in the incidence of permanent facial nerve injury (2 versus 1 per cent respectively). This study demonstrates that dissection in close proximity to the tumour is possible without inducing recurrence and that in practice the microinvasion of the capsule by tumour buds has limited clinical significance.

Adenoma, Pleomorphic↗

PubMed2005

Fine-needle aspiration biopsy of cystic pleomorphic adenoma with adnexa-like differentiation mimicking mucoepidermoid carcinoma: a case report.

Although pleomorphic adenomas (PAs) usually can be diagnosed very accurately with fine-needle aspiration biopsy (FNAB), even this common salivary gland neoplasm can be diagnostically challenging and cause pitfalls in cytodiagnosis. In particular, the presence of either cystic changes or squamous, mucinous, or sebaceous metaplasia can lead to a false positive diagnosis of mucoepidermoid carcinoma (MEC). Here, we present a case of a 70-yr-old man with an asymptomatic left deep lobe parotid mass for which CT-guided FNAB was performed. The FNAB cytology revealed cohesive clusters of squamous epithelial cells, sebaceous cells, oncocytes, macrophages, and rare myoepithelial cells. Characteristic metachromatic fibrillar chondromyxoid stroma, which usually is seen in PAs, was not seen in the aspirate. Although cytodiagnosis of PAs was suggested based on the presence of other cellular components, resection was recommended. The subsequent parotidectomy specimen revealed an encapsulated cystic PA with mixed appendageal differentiation including areas of squamous, mucinous, sebaceous, and oncocytic metaplasia. Chondromyxoid stroma was only focally present. Presence of squamous, mucinous, and/or sebaceous metaplasia, especially in the absence of chondromyxoid stroma, presents the potential for misinterpretation of the FNAB as indicative of malignancy in general and MEC in particular.

Adenoma, Pleomorphic↗

PubMed2005

Spontaneous infarction of a pleomorphic adenoma in parotid gland: diagnostic problems and review.

Although infarction of parotid gland pleomorphic adenoma (PA) following fine-needle aspiration (FNA) has been well-documented, spontaneous infarction of PA has remained as an uncommon entity in the literature. To our knowledge, we report the second case of spontaneous infarction occurring in a parotid gland PA. A 44-yr-old man presented with a 2-yr history of slowly enlarging right parotid mass, which had become painful 1 mo before performing FNA biopsy. Smears revealed abundant necrotic debris, atypical squamous cells, and small cells with dark nuclei suggestive of a carcinoma. Histologic examination of the tissue fragments demonstrated degenerated clusters of cells and chondromyxoid matrix. The parotidectomy specimen had features consistent with those of an infarcted PA. Although appears to be rare, spontaneous infarction of PA should be considered in the differential diagnosis, since necrosis may mimic carcinoma and cause misinterpretation of necrosis as an indication of malignancy, in an otherwise benign salivary gland neoplasm.

Adenoma, Pleomorphic↗

PubMed2005

Pleomorphic adenoma of the breast: case report and review of the literature.

Pleomorphic adenoma of the breast (PAB) is a very rare neoplasm. Although quite unique in its morphology, PAB shares some similarities with adenomyoepithelioma and is considered by some authors as a variant of this entity. Cytologic diagnosis of this lesion can be very challenging, especially when limited sampling is available. The differential diagnosis of PAB includes metaplastic carcinoma. On cytologic material, fibroadenoma and phyllodes tumor should also be considered within the differential diagnosis. We report the findings in a case of PAB, initially misdiagnosed as mucinous carcinoma on fine-needle aspiration, and review the literature regarding this entity. Correct identification of this benign mammary neoplasm is important to avoid unnecessarily aggressive treatment.

Adenocarcinoma, Mucinous↗