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PubMed2002

Pleomorphic hyalinizing angiectatic tumor of soft parts: a case report and literature review.

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare, recently recognized neoplasm occurring predominantly in the subcutaneous tissue of the lower limbs of adults. We report a case of PHAT in an 83-year-old woman who presented with a 5.0 x 5.0 x 2.0 cm mass in the soft part of her left thigh. Histologically, the tumor was well circumscribed by a thin fibrous capsule and predominantly composed of fusiform cells with eosinophilic cytoplasm and round-to-oval or pleomorphic nuclei. The tumor cells resembled those of malignant fibrous histiocytoma, but differed from them by less prominent mitotic figures. Immunohistochemically, the tumor cells were diffusely and strongly positive for CD34; partially positive for vimentin and CD99 (MIC-2); and negative for epithelial and non-epithelial markers. Ultrastructurally, the tumor cells had pleomorphic cytoplasm and nucleus. Intermediate-sized cytoplasmic filaments were observed in a few tumor cells, but neurosecretory-type granule-like intracytoplasmic organelles were not seen. These findings suggest that this tumor is derived from stromal fibroblast, such as solitary fibrous tumors or giant cell angiofibroma.

12E7 Antigen↗
PubMed2000

Pleomorphic lipoma: case report and literature review.

BACKGROUND: Pleomorphic lipoma is a rare, benign, pseudosarcomatous, soft tissue neoplasm typically involving the subcutis of the neck and shoulder in middle-aged to elderly men. It is characterized histologically by atypical, multinucleated giant cells and grossly as a well-circumscribed mass. Since this neoplasm can resemble a sarcoma, histopathologic diagnosis is critical in preventing unnecessary surgery. OBJECTIVE: To describe a case of pleomorphic lipoma in the neck and to review the clinical and histopathologic characteristics of this neoplasm. METHODS: We present a case report and review of the literature. RESULTS: Local excision to completely extirpate this neoplasm has proven curative at 10 months of follow-up. CONCLUSION: Pleomorphic lipoma is superficially located and may be encountered in a dermatologic setting. Thus the dermatologist should become familiar with its clinical presentation and microscopic appearance.

Aged↗
PubMed2002

Systemic amyloidosis associated with pleomorphic sarcoma of the spleen and remission of nephrotic syndrome after removal of the tumor.

We report a case of a 57-year-old woman who was diagnosed with a systemic AA amyloidosis associated with a pleomorphic sarcoma of the spleen. Although the association and causality between chronic inflammatory states and systemic AA amyloidosis have been well established, the evidence linking solid malignancies to reactive AA amyloidosis is scarce. Our patient had a significant systemic amyloid deposition including biopsy-proven renal and cardiac AA amyloidosis. Subsequent evaluation uncovered the presence of a large splenic mass, which was treated by splenectomy. Histologically the splenic tumor was classified as pleomorphic sarcoma. The removal of the tumor resulted in a marked decline in proteinuria, stable renal and cardiac functions, and symptomatic improvement at 1-year follow-up. Based on the noted improvements, we speculate that the pleomorphic sarcoma of the spleen caused secondary amyloidosis in our case. Stromal tumors, although rare, may be associated with and should be considered in the differential diagnosis of the cause of AA amyloidosis.

Amyloidosis↗
PubMed2002

Pleomorphic rhabdomyosarcoma in an adult forearm: a case report.

Pleomorphic rhabdomyosarcoma (RMS) is a rare malignancy, and treatment protocols for this tumor are not established. Although Intergroup Rhabdomyosarcoma Study data have led to improvement in overall and disease-free survival for children with juvenile RMS, the applicability of those findings to adult pleomorphic RMS is uncertain. This report presents the case of a 29-year-old man with pleomorphic RMS of the forearm (a location not previously reported in current literature); reviews the immunohistochemical, ultrastructural, and histologic diagnostic criteria; and discusses treatment.

Adult↗
PubMed2000

Highly pleomorphic staphylococci as a cause of cancer.

An extensive historical literature exists suggesting that bacteria and other non-virus microorganisms cause cancer. Much of this literature stresses the likely involvement of highly pleomorphic bacteria in carcinogenesis. Pleomorphic bacteria exhibit a variety of morphological types, some of which are identical to other bacteria. In particular, bacteria that can express more than one morphology, including that normally associated with common species of Staphylococcus, have frequently been isolated from cancers. Not surprisingly, this has led to considerable confusion and ridicule. The literature linking highly pleomorphic bacteria with carcinogenesis is presented here in an attempt to add weight to the view that bacteria, notably those expressing the morphology of common species of staphylococci, cause cancer.

Humans↗
PubMedDate not supplied

Fibronexus in "malignant fibrous histiocytoma" of the bone: a case report of pleomorphic myofibrosarcoma.

The fibronexus (fibronexus junction) has been thought to be a characteristic ultrastructural feature of myofibroblasts, but it is controversial whether the fibronexus is also a characteristic of various myofibroblastic tumors. We report here a case of pleomorphic bone sarcoma (pleomorphic/storiform malignant fibrous histiocytoma) with fibronexus junctions arising in the head of the left humerus of a 70-year-old woman. By light microscopy the tumor was composed of large spindle or polygonal cells occasionally arranged in fascicles. Foamy giant cells with bizarre nuclei were not uncommon. Immunohistochemically, the tumor cells were positive diffusely for vimentin and focally for muscle actin (HHF35) and alpha-smooth muscle actin but were negative for desmin, high-molecular weight caldesmon, and S-100 protein. Ultrastructurally, the tumor cells had indented nuclei and spindle or polygonal cytoplasm, with little rough endoplasmic reticulum and small vesicles, and rather numerous mitochondria. The tumor cells had myofilaments with focal densities in the periphery, fibronectin fibrils adjacent to intracellular myofilaments, and by definition, therefore, fibronexus junctions. These findings suggest myofibroblastic differentiation, and a diagnosis of pleomorphic myofibrosarcoma is thought to be appropriate. We believe that the fibronexus is a characteristic and useful ultrastructural feature for differentiating myofibroblastic tumors from other pleomorphic myogenic sarcomas.

Actin Cytoskeleton↗
PubMed1996

Pleomorphic (giant and spindle cell) carcinoma is genetically distinct from adenocarcinoma and squamous cell carcinoma by K-ras-2 and p53 analysis.

Pleomorphic carcinoma (PC) of lung is a poorly differentiated epithelial neoplasm predominantly composed of pleomorphic giant and/or spindle tumor cells. The WHO classification of lung cancer recognizes spindle cell carcinoma and giant cell carcinoma as separate neoplasms related to squamous cell carcinoma (SqC) and large cell carcinomas, respectively. Further, the presence of foci of SqC or adenocarcinoma (AdC) in, respectively, 10% and 45% of PC produces additional uncertainty as to the distinctive nature of this tumor type. In this study, the authors tested the hypothesis that PC is an entity separate from SqC or AdC by evaluating the mutational spectrum seen in these tumor types. This is performed by documenting and comparing mutation type and rate of K-ras-2 and p53 genes in PC, SqC, and AdC. Comparative DNA sequence and immunohistochemical analysis were performed on 22 PC, 42 SqC, and 97 AdC. Archival formalin-fixed, paraffin-embedded tissues formed the basis of the study. Immunohistochemical staining with p53 antibody (DO-7) revealed statistically significant differences in the intensity and frequency of staining of PC (weak, 86% of cases) versus SqC (strong, 52% of cases) and AdC (strong, 27% of cases) (P < .001). Topographic genotyping with subsequent polymerase chain reaction (PCR) and sequence analysis of K-ras-2 showed mutations in significantly fewer cases of PC (9%, 2 of 22 cases) than in AdC (36%, 35 of 97 cases) or SqC (0%, 0 of 42 cases) (P < .001). Pleomorphic carcinoma also showed significantly fewer p53 point mutations (14%, 3 of 22 cases) than did AdC (27%, 26 of 97 cases) of SqC (43%, 18 of 42 cases) (P < .01). Finally, the p53 mutations in PC were more common in exon 7, whereas those in SqC and AdC were more frequent in exon 8. These findings reveal significant differences in the pattern and frequency of genetic mutations between PC and pulmonary SqC and AdC and are in keeping with the separate histopathologic classification of these tumors.

Adenocarcinoma↗
PubMed1988

Spindle-cell and pleomorphic neoplasms of the skin. A clinicopathologic and immunohistochemical study of 30 cases, with emphasis on "atypical fibroxanthomas".

Atypical fibroxanthoma belongs to the family of spindle-cell and pleomorphic neoplasms of the skin. The lineage of differentiation of this tumor and the means whereby it can be diagnostically separated from other similar morphologic entities have been controversial. In order to address these issues, the authors studied 30 spindle-cell and/or pleomorphic cutaneous tumors, including atypical fibroxanthomas (AFXs), superficial malignant fibrous histiocytomas (MFHs), dermatofibrosarcoma protuberans (DFSPs), sarcomatoid squamous-cell carcinomas (SCCs), spindle-cell malignant melanomas (MMs), and leiomyosarcomas, (LMSs). These cases were analyzed using a panel of eight antibodies and the immunoperoxidase technique. AFXs were reactive for vimentin, alpha-1-antichymotrypsin (AACT), alpha-1-antitrypsin (AAT), and cathepsin-B (CB) but failed to display cytokeratin (CK), epithelial membrane antigen (EMA), S-100 protein, and desmin. MFHs and DFSPs exhibited immunophenotypic profiles that were nearly identical to that just described. In contrast, SCCs were typified by positivity for CK and EMA; MMs exhibited uniform reactivity for S-100 protein; and LMSs contained desmin in four of five cases. A surprising result was the expression of S-100 by LMSs. Also, all tumors displayed at least one of the three proteolytic enzymes assessed in this study (AAT, AACT, and CB), demonstrating the relative diagnostic nonspecificity of these determinants. It is concluded that AFXs are "fibrohistiocytic" neoplasms, with substantial morphologic and immunohistochemical similarity to MFHs. The immunohistochemical classification of spindle-cell and pleomorphic tumors of the skin necessitates the use of antibody panels to assess the presence of markers that are characteristic of each diagnostic group.

Adolescent↗
PubMed1991

Primary cutaneous pleomorphic T-cell lymphoma expressing CD30 antigen.

Pleomorphic peripheral T-cell lymphomas (PPTL) arising primarily in the skin are uncommon. The observation of pleomorphic T-cell lymphoma composed of 100% CD30+ cells is rare. We report the case of a woman in whom such a tumor manifested itself with a single cutaneous lesion. It was composed of medium to large cells expressing markers of helper/inducer T-lymphocytes, as well as activation (CD30) and proliferation antigens (Ki-67), but not the CD25 activation antigen. The lesion was excised and the patient is alive and disease free 2 years after the diagnosis. This case confirms previous reports about the favorable course of some cutaneous pleomorphic lymphomas, and shows that the activation antigen expression and proliferation antigen (Ki-67) expression do not seem to worsen the prognosis.

Antigens, CD↗
PubMed1997

Apoptosis in atypical fibroxanthoma and pleomorphic malignant fibrous histiocytoma.

Explanations for the disparate behavior of atypical fibroxanthoma (AFX) as compared with pleomorphic malignant fibrous histiocytoma (MFH) have included the proposition that the former is a pseudosarcoma. Nonetheless, these tumors are now widely regarded as the same process, but with AFX behaving benignly by virtue of its superficial location. However, a neoplasm's metastastatic potential has been proposed to be related to apoptosis. Therefore, the aim of the present study was to examine apoptotic counts, in conjunction with two important regulators of apoptosis: p53 and bcl-2, to determine if a distinction exists that may account for the different outcomes of these lesions. There was no significant statistical difference between eight AFX and nine pleomorphic MFH in terms of apoptotic behavior, proliferative indexes, p53 protein expression, or presence of bcl-2 product. Therefore, our results further support the contention that AFX should be regarded as a form of pleomorphic MFH, which demonstrates low malignant potential by virtue of its location in readily accessible sites.

Adult↗
PubMed1998

Subungual myxoid pleomorphic fibroma.

The authors report an example of pleomorphic fibroma in a unique site: the subungual space of the thumb. A 54-year-old man presented with paronychia and markedly thickened hyperkeratotic nail. The nail plate was removed, and an exophytic, red, friable, granulation tissue-like lesion was revealed subungually. The lesion was diagnosed clinically as pyogenic granuloma. Excisional biopsy was performed. Histologically, the lesion showed a hypocellular fibroma with myxoid areas and dilated blood vessels. Interspersed with ordinary appearing fibroblasts, there were bizarre, atypical fibroblasts with hyperchromatic and large, pleomorphic nuclei and multinucleated floret-like giant cells. These atypical cells showed strong immunoreactivity with antibodies against CD34 and vimentin. Occasional positivity was noted with factor XIIIa and alpha-1-antichymotrypsin, whereas no reactivity occurred with alpha-1-antitrypsin, actin, or S-100 protein. The entity of pleomorphic fibroma has been reported in many body sites; however, this is the first case to be reported in the subungual space.

Antigens, CD34↗
PubMed2000

Pleomorphic angioleiomyoma.

We describe two cases of pleomorphic angioleiomyoma. In one case, a 46-year-old man presented with a single nodule on his scrotum of 1 year's duration, and in another, a 38-year-old woman presented with a single nodule on her right knee of 1 year's duration. In both cases, histopathologic examination showed a well-circumscribed nodule composed of smooth muscle and numerous veins and capillaries. Contrary to the ordinary angioleiomyoma, marked nuclear pleomorphism was noted. Although mitoses were rare, immunohistochemistry revealed many tumor cells that were positive for proliferating cell nuclear antigen, Ki-67, and p53, indicating that the pleomorphic appearance does not simply represent a degenerative change of some tumor cells.

Adult↗
PubMed1999

Pleomorphic xanthoastrocytoma of the retina.

We report two cases of pleomorphic xanthoastrocytomas of the retina. The immunoreactivity to CD68, Fe (Pearls' technique), astrocytes (argentic technique), and antibodies to glial fibrillary acidic protein was tested in tumor cells. Both neoplasms contained cells immunoreactive for glial fibrillary acidic protein and CD68. These tumors, observed in the CNS, first reported by Kepes et al. were found in the retina as well-circumscribed masses with a cystic component. Many large pleomorphic cells contained vesicular and enlarged nuclei with a homogeneous eosinophilic cytoplasm, prominent nucleoli, and calcium deposits. Some cells had a markedly swollen lipidized cytoplasm, whereas others were spindle-shaped and contained multiple nuclei. These findings suggest that our two specimens have an astroglial lineage and are similar to pleomorphic xanthoastrocytomas of the CNS brain and spinal cord. Both reported cases were in female patients who were in their 20s and had glaucoma. After 10 years both are free of disease.

Adult↗
PubMed2002

Pleomorphic liposarcoma metastatic to the thyroid gland.

Although carcinoma is rarely metastatic to the thyroid gland, it has an established place in the differential diagnosis of cold nodules on radionuclide thyroid scans in patients with known cancers. A case of metastatic pleomorphic liposarcoma with involvement of the thyroid gland adds to the list of cases of metastases to the thyroid gland. A patient with known metastatic pleomorphic liposarcoma with a palpable thyroid nodule of 2 months' duration had a thyroid scan with 130 MBq (3.5 mCi) Tc-99m pertechnetate. The scan showed a cold nodule occupying most of the right lobe of the thyroid. Cytologic analysis of a fine-needle aspirate from this cold nodule confirmed metastasis from the known primary cancer, pleomorphic liposarcoma. In a patient with a known primary tumor, a nodule in the thyroid gland should be considered a metastatic lesion, regardless of the time since the primary tumor was diagnosed and treated, until proved otherwise.

Biopsy↗
PubMed2002

Intrasellar pleomorphic xanthoastrocytoma: case report.

OBJECTIVE AND IMPORTANCE: A patient with a primary intrasellar pleomorphic xanthoastrocytoma is described. CLINICAL PRESENTATION: A 78-year-old woman experienced slowly progressing bilateral visual disturbance. Analysis of magnetic resonance imaging scans indicated a well-enhanced tumor occupying intra- and suprasellar spaces with displacement of the pituitary gland anteriorly. INTERVENTION: Partial resection of the tumor via the transsphenoidal route brought about improvement of the patient's visual disturbance. The tumor was conspicuously pleomorphic and composed of plump to spindle-shaped large and bizarre cells with single or multiple nuclei and lipid-laden foamy cytoplasm. The neoplastic cells were positive for glial fibrillary acidic protein, S-100 protein, and vimentin. The MIB-1 antibody labeling index was very low (<1%) CONCLUSION: Pleomorphic xanthoastrocytoma should be included in the differential diagnosis of tumors arising in the posterior hypophysis.

Aged↗
PubMed2005

Primary cutaneous myxofibrosarcoma mimicking pleomorphic hyalinizing angiectatic tumor (PHAT): a potential diagnostic pitfall.

Myxofibrosarcoma (MFS) is one of the most common sarcomas of adults, and includes lesions ranging from low to high grade based on increasing cellularity, nuclear pleomorphism, and mitotic activity. We present an unusual case of MFS, which initially showed features of a pleomorphic hyalinizing angiectatic tumor (PHAT), a rare soft-tissue tumor considered benign in the WHO classification. The initial lesion showed a subcutaneous proliferation of spindled to polygonal eosinophilic cells with striking cellular pleomorphism, set in a myxoid to sclerotic stroma with prominent hyalinized and angiectatic vasculature, classic characteristics of PHAT. Rare mitotic figures were identified. The preliminary diagnosis of PHAT was confirmed by a consultant expert soft-tissue pathologist. Four months after the initial surgery, local recurrence was noted. The recurrent tumor demonstrated findings of a high-grade MFS, with a diffuse and cellular proliferation of atypical spindle cells set in a prominent myxoid stroma. Multiple mitoses, including atypical ones, were present. In light of these findings, the original specimen was reexamined and the initial diagnosis was amended to MFS. MFS may mimic or be confused with several benign soft-tissue lesions. Cases mimicking PHAT have not been previously reported. We describe a unique case of a high-grade MFS that initially showed characteristic features of a PHAT, to illuminate the fact that MFS may rarely arise in, or have areas that masquerade as, PHAT. It is also conceivable, although speculative, that at least some cases of PHAT, currently considered a benign tumor by the WHO, may actually represent or evolve into a unique form of MFS of low malignant potential.

Aged↗
PubMed2004

Pleomorphic liposarcoma: clinicopathologic analysis of 57 cases.

Pleomorphic liposarcoma is an uncommon form of liposarcoma that only recently has been properly characterized. A series of 57 cases is presented. Patient age at presentation ranged from 27 to 95 years (median, 54 years), and there was a slight male predilection (male/female ratio = 1.2:1). Tumors most frequently involved the lower limb (47% of cases) or upper limb (18%). Other anatomic sites, including trunk (14%), retroperitoneum (7%), head and neck (5%), abdomen/pelvis (5%), and spermatic cord (4%), were less frequently involved. Tumor size ranged from 1.5 to 21 cm (median, 8 cm), with deep (subfascial) locations (39 cases) being more frequent than subcutaneous (11 cases) or dermal sites (5 cases). All lesions showed features of pleomorphic sarcoma and at least focally contained typical multivacuolated lipoblasts. Although there was considerable overlap, tumors fell into three broad categories: high-grade pleomorphic/spindle cell sarcoma with scattered lipoblasts or sheets of lipoblasts (60%), high-grade pleomorphic sarcoma with epithelioid areas and scattered lipoblasts (28%), and intermediate- to high-grade sarcoma predominantly resembling myxofibrosarcoma except for the presence of lipoblasts (12%). Immunohistochemistry revealed focal staining for smooth muscle actin in 13 of 29 cases (45%), S-100 protein positivity in lipoblasts in 15 of 45 cases (33%), focal staining for keratin in 6 of 28 cases (21%), including 5 of 13 (38%) with epithelioid morphology, and focal staining for desmin in 4 of 30 cases (13%). Follow-up data, available in 50 patients (88%) (median, 33 months), showed local recurrence in 34% of patients, systemic metastases in 32%, and tumor-related death in 32%. Only 2 of the 16 superficial (dermal or subcutaneous) lesions metastasized. Five-year overall, local recurrence-free, metastasis-free, and disease-free survivals were 63%, 58%, 58%, and 39%, respectively. By univariate analysis, central (nonextremity) location, deep situation, tumor size > or =10 cm, mitotic rate > or =10 per 10 HPF, necrosis, and epithelioid morphology were associated with a worse prognosis. However, by multivariate analysis, only age > or =60 years, central location, tumor size, and mitotic rate remained independent predictors for an adverse outcome. By multivariate analysis, wide local excision or amputation and postoperative radiotherapy protected against local recurrence.

Adult↗
PubMed2004

Atypical pleomorphic epithelioid angiomyolipoma localized to the pelvis: a case report and review of the literature.

AIMS: Angiomyolipoma is the most common mesenchymal tumour of the kidney. It has been reported in several other sites outside the kidney, mainly in the liver. We report the first case of atypical pleomorphic angiomyolipoma in a man, arising from the pouch of Douglas and extending to the entire abdominal cavity. METHODS: A 17-year-old man underwent a complete resection of a giant abdominopelvic mass. The tissue was formalin fixed and paraffin embedded and 4 micro m thick histological sections were stained with haematoxylin-eosin. Immunohistochemical stains for HMB-45, smooth muscle actin, vimentin, calponin, S100 and desmin were performed. Sections for electron microscopy were also prepared. RESULTS: Microscopic examination revealed a neoplasm composed of pleomorphic epithelioid cells with atypical features, immunoreactive for HMB-45, MART-1, actin, vimentin and calponin, while S100 protein and desmin stains were negative. Ultrastructurally, the tumour cells showed prominent nucleoli, vacuolated cytoplasm, and some premelanosomes. A diagnosis of atypical pleomorphic epithelioid angiomyolipoma was then made. CONCLUSIONS: To date five patients with abdominal epithelioid angiomyolipoma have been described in the literature. All were women. Three of the five patients reported developed metastasis, while our patient is still free of disease at 16 months of follow-up. Clear prognostic pathological features have not been identified.

Adolescent↗