Angiectatic Tumor Of Soft Parts Research Articles

A Subcutaneous Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts of the Right Ankle: A Case Report

Abstract- Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare, slow-growing mass, which is locally aggressive and classified as tumors of borderline malignancy. In this study, we report a case of subcutaneous ankle mass in 43-year-old female since 2 years ago with a pathological diagnosis of PHAT, characterized by clusters of ectatic, fibrin-lined, and thin-walled vessels which are surrounded by a spindled or pleomorphic neoplastic stroma.

Treatment modalities and outcomes of pleomorphic hyalinizing angiectatic tumor: a systematic review of the literature

A systematic review of the cases documented in the literature regarding Pleomorphic hyalinizing angiectatic tumor of soft parts (PHAT) was performed in order to identify (1) location on presentation (2) surgical treatment modality (3) recurrence rate (4) any associations between location, age, histology, surgery type on recurrence. A systematic review of medical literature listed on PubMed was conducted identifying any prior case report and/or case series of diagnosed PHAT, with no exclusion based on language or time. Twenty-nine articles were identified removing any articles with duplicated cases yielding a total of 93 cases. Cases were broken down by gender, presenting location (UE/LE/axial), surgery type [wide local resection, non-wide local, wide local with radiation therapy (RT), non-wide local with RT], recurrence, and time to recurrence. The mean age at presentation was 54.5±17.1 (range 10-89) with the 76% of cases appearing in the lower extremity (15% UE, 9% Axial). Of the 93 patients, 74 had a known surgical procedure, 31% WL, 40% NWL, 8% WL+RT, 1% NWL+RT. Of those treated surgically, 63 pts had documented follow-up and 18 (29%) had recurrence. A strong association was observed between surgery type and recurrence. Local recurrence was more common within the group undergoing NWLE in 52% (16/41) of cases (p=0.002). Kaplan-Meier analysis showed an estimate mean time for recurrence of 43.87months [95% confidence interval (CI) 24.52-63.22; and standard error (SE) 7.59] for the entire population. A trend was also seen toward males having a shorter disease-free survival than females (29.4 mos. vs. 69.5 mos.). No significant association seen between size, location, histology type and recurrence. PHAT has a characteristic presentation in the LE with a relatively high rate of local recurrence and slow-growing potential. Wide local excision appears to be superior in decreasing recurrence rates and a long-term follow-up period is needed.

Pleomorphic hyalinizing angiectatic tumor of soft parts with unusual lipoma-like clinical morphology

Pleomorphic hyalinizing angiectatic tumor of soft parts with unusual lipoma-like clinical morphology

Pleomorphic hyalinizing angiectatic tumor of soft parts: Case Report with unusual ganglion-like cells and review of the literature

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a recently described, non-metastasizing tumor of uncertain lineage. This tumor distributes equally between the genders and has a predilection for the subcutaneous soft tissue, particularly in lower extremity, other locations are rare. Based on the recent literature, PHAT is suspected to encompass the morphological spectrum with other tumors such as myxoinflammatory fibroblastic sarcoma (MIFS) and hemosiderotic fibrolipomatous tumor (HFLT), although cytogenetic data remain inconsistent. We report a case of PHAT that arose in the upper arm with unusual morphology which showed ganglion-like cells similar to Reed-Sternberg-like cells found in MIFS. The tumor had strong immunohistochemical expression of CD34, CD99, and was negative for S-100. The ganglion-like cells were positive for both CD34 and CD68 but negative for CD30. The translocation between chromosome 1 and 10, a frequent finding of MIFS and HFLT, was not identified by FISH excluding the possibility of hybrid PHAT and MIFS. We conclude FISH can be a potential useful tool to separate PHAT with atypical morphology from hybrid tumor in doubted cases. Due to the rarity of PHAT and lack of consistent pathogenetic signatures, more cases and further studies will be needed to elucidate the pathogenesis and nature of this tumor.

English

English

Complex analysis of a recurrent pleomorphic hyalinizing angiectatic tumor of soft parts

Pleomorphic hyalinizing angiectatic tumor of soft parts is a recently recognized, low-grade mesenchymal neoplasm of uncertain lineage. It is characterized by clusters of ectatic, fibrin-lined, thin-walled vessels surrounded by pleomorphic but mitotically inert spindled cells with frequent intranuclear inclusions and a variable inflammatory component. Here, we report a case of recurrent pleomorphic hyalinizing angiectatic tumor in a 37-year-old white woman. Touch imprint cytologic analysis revealed oval and spindled cells with fine chromatin, occasional prominent intranuclear inclusions, and intracytoplasmic pigment. The histologic features of the cells constituting the bulk of tumor were those characteristic of a putative precursor lesion ("early pleomorphic hyalinizing angiectatic tumor") as well as another recently described entity known as hemosiderotic fibrohistiocytic lipomatous lesion. Cytogenetic analysis revealed 2 unbalanced translocations involving chromosomes 1 and 3 and chromosomes 1 and 10, with a karyotype of 45,XX,der(1)t(1;3)(p31;q12),-3,der(10)t(1;10)(p31;q25)[11]/46,XX[4], thus suggesting that this disease is neoplastic. The controversies in classification of these lesions are also discussed.

Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts

Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts is a rare, nonmetastasizing tumor of uncertain lineage which was first reported in 1996. Here, we report a case of PHAT and review the literature. A 49-year-old man presented with a soft and progressively enlarging mass over the right buttock for several years. On suspicion that the mass was a right gluteal lipoma, he underwent surgical excision. The excised lesion measured 14 x 6 x 3.5 cm. It had a variegated appearance with a white-tan to yellowish color on the cut surface. Some punctate hemorrhage and vessel thrombosis were seen. Microscopically, the tumor was a PHAT characterized by clusters of ectatic, fibrin-lined, thin-walled vessels, which were surrounded by a mitotically inert, spindled, pleomorphic, neoplastic stroma that contained a variable inflammatory component. Immunohistochemical study showed that the tumor cells were positive for CD34, and negative for S-100, HMB45 and actin. The patient experienced local recurrence 6 months later. The recurrent tumor was widely excised. No evidence of metastasis was found during the 18 months after the second operation. The recurrent lesion had a microscopic appearance that was similar to the initial lesion.

Pleomorphic hyalinizing angiectatic tumor of soft parts involving the upper limb

Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts is a low grade, rare, soft tissue tumor which commonly occurs on the lower extremities of adults of either sex, though lesions in other anatomic locations have been described. We present a case of a 65-year-old female patient who presented with a left forearm swelling and underwent a wide local excision. Histopathology revealed it to be a PHAT.

Pleomorphic hyalinizing angiectatic tumor of soft parts:a case report

Pleomorphic hyalinizing angiectatic tumor of soft parts:a case report

CD34‐reactive tumors of the skin. An updated review of an ever‐growing list of lesions

Over the past few years, a growing number of cutaneous tumors expressing CD34 is being reported. The list contains benign and malignant neoplasms as well as reactive and hamartomatous lesions of diverse lineages of differentiation, including fibroblastic, myofibroblastic, fibrohistiocytic, vascular, neural, adipocytic, smooth muscle, hematopoietic, melanocytic and epithelial. The more frequent diagnostic difficulties are found in spindle cell proliferations, mainly in those of the fibrocytic lineage. In part, this is because of the fact that in this area are, aside to well-defined entities, histologically and clinically diverse, recently reported cutaneous CD34-reactive lesions, whose definitions, limits and relationships are not completely established. The CD34 expression plays a key role in the differential diagnosis of some tumors, such as dermatofibrosarcoma protuberans, epithelioid sarcoma or pleomorphic hyalinizing angiectatic tumor of soft parts, with important therapeutic consequences. In others, as in desmoplastic trichilemmoma, it can help to resolve diagnostic problems in concrete cases. Finally, in many of the CD34-positive lesions, the diagnosis with the hematoxylin and eosin stain is straightforward. However, in all of them, the knowledge of the immunohistochemical profile contributes to our understanding of the cutaneous pathology.

THIS ARTICLE HAS BEEN RETRACTED CD34‐reactive tumors of the skin. An updated review of an ever‐growing list of lesions

Over the past few years, a growing number of cutaneous tumors expressing CD34 is being reported. The list contains benign and malignant neoplasms as well as reactive and hamartomatous lesions of diverse lineages of differentiation, including fibroblastic, myofibroblastic, fibrohistiocytic, vascular, neural, adipocytic, smooth muscle, hematopoietic, melanocytic and epithelial. The more frequent diagnostic difficulties are found in spindle cell proliferations, mainly in those of the fibrocytic lineage. In part, this is because of the fact that in this area are, aside to well‐defined entities, histologically and clinically diverse, recently reported cutaneous CD34‐reactive lesions, whose definitions, limits and relationships are not completely established. The CD34 expression plays a key role in the differential diagnosis of some tumors, such as dermatofibrosarcoma protuberans, epithelioid sarcoma (ES) or pleomorphic hyalinizing angiectatic tumor of soft parts, with important therapeutic consequences. In others, as in desmoplastic trichilemmoma, it can help to resolve diagnostic problems in concrete cases. Finally, in many of the CD34‐positive lesions, the diagnosis with the hematoxylin and eosin stain is straightforward. However, in all of them, the knowledge of the immunohistochemical profile contributes to our understanding of the cutaneous pathology.

Clinicopathologic features of pleomorphic hyalinizing angiectatic tumor of soft parts

Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts is a rare soft tissue tumor, which is generally considered low-grade. To distinguish the tumor from other soft tissue lesions, we analyzed the clinicopathologic and ultrastructural features, immunophenotypes, and flow cytometric DNA ploidy of PHAT in 9 cases. PHAT specimens were collected from 9 patients with PHAT from 1990 to 2004. Each specimen was cut into pieces and stained with hematoxylin-eosin, phosphotungstic acid-hematoxylin, Prussian blue, and Masson trichrome, respectively. Immunohistochemical stains for vimentin, S-100 protein, CD34, CD31, CD99, VEGF, desmin, CD117, alpha-SMA, and MIB-1 were performed with the Envision system. Flow cytometry was used in four specimens, two of which were observed by electron microscopy. In the 9 cases, the PHAT occurred at the lower extremity in 2 patients, inguinal in 2, waist in 1, forearm in 1, buttock in 1, foot in 1, and the chest wall in 1. All the lesions presented in the superficial subcutaneous tissues. Follow-up data were available in 7 of the patients, among whom 2 (28.6%) had recurrence after primary therapy. Microscopically, typical PHAT was characterized by sheet-like proliferation of spindle or pleomorphic cells and clusters of thin-walled hyalinized cstatic vessels. In some areas of the tumor, hemosiderin-laden spindle cells, numerous small single vessels, and myxoid extracellular matrix could be identified, indicating an "atypical PHAT". Mitotic figures were rare in all the cases. In 5 of the 9 patients (55.6%), the tumor was typical PHAT; and in the other 4 (44.4%), typical and atypical PHAT coexisted. Immunohistochemically, the neoplastic cells were positive for vimentin, CD34, CD99, and VEGF, but negative for S-100 protein, desmin, SMA, and CD31. In all the cases, the MIB-1 proliferative activity of the neoplastic cells was lower than 2%. Ultrastructural analysis did not reveal any evidence of specific differentiation. Aneuploidy was not detected by flow cytometry. Histologically, typical PHAT is characterized by spindle and pleomorphic cells associated with an angiectatic vasculature. The neoplastic cells often express vimentin and CD34, and may be positive for CD99 and VEGF. Ultrastructurally, the tumor usually has no specific differentiation. The low MIB-1 index and the absence of aneuploidy in PHAT indicate a non-malignancy. However, we consider the tumor as a borderline neoplasm because of its aggressive behaviour, and suggest wide local resection with tumor-free margin for the treatment of the disease.

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

Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts is a rare soft tissue neoplasm. Few cases have been reported in the literature thus far. PHAT is a spindle cell neoplasm with a prominent vascular component which displays some features of both neurilemoma and malignant fibrous histiocytoma. Lesions typically occur on the lower extremities of adults, however, lesions in other anatomic locations have been described. In this paper, we report a 60 years old female with PHAT of the right foot and review the current literature. El-Tal A-E-K, Mehregan D. Pleomorphic hyalinizing angiectatic tumor of soft parts: case report and literature review.

Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts: Ultrastructural Analysis of a Case with Original Features

Pleomorphic hyalinizing angiectatic tumor, a rare neoplasm of uncertain lineage resembling malignant fibrous histiocytoma and schwannoma, was first described in 1996 by M. E. F. Smith et al. (Am Surg Pathol. 20:21–29). To date, less than 100 cases have been reported in the international literature. It occurs in subcutaneous and intramuscular soft tissues of extremities or trunk in adults without sex predilection. All lesions are composed of sheets and fascicles of spindled and pleomorphic cells associated with clusters of thick-walled ectatic vessels surrounded by a perivascular hyaline material and inflammatory cells such as mast cells. About one-half of these neoplasms express CD34. No patient has developed metastases but occasional local recurrences are possible. This tumor of uncertain lineage is suggested to be an aggressive locally growing low-grade sarcoma. Only 3 cases were previously studied by electron microscopy and appeared to consist of primitive fibroblastic cells. The authors report histological and ultrastructural characteristics of a new case of PHAT excised from the right buttock of a 66-year-old man with the presence of ganglion-like cells, a feature that has not been previously reported, and unusual central ischemic necrosis. The features of this case are suggestive of a fibroblastic origin.

Pleomorphic hyalinizing angiectatic tumor of soft parts

A 63-year-old morbidly obese man presented with a 4-year history of right breast enlargement. The right breast had been slowly growing in size until several months before surgery, when the rate of growth dramatically increased. Physical examination revealed massive enlargement of the right breast (Fig 1). Preoperative magnetic resonance imaging (MRI) showed a 25.6(W) × 18.3(AP) × 26.3(CC) cm well-defined mass within the right breast. It was predominantly cystic and had a suggestion of an enhancing soft tissue component, measuring 10.7(W) × 3.9(AP) × 8.3(CC) cm at the anteromedial aspect of this mass (Fig 2). The mass did not appear to involve the pectoralis major muscle radiologically.

Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts

Abstract We report morphologic, flow cytometric, and immunohistochemical findings in two cases of pleomorphic hyalinizing angiectatic tumor of soft parts. Both patients were middle-aged women with subcutaneous lesions located in the lower extremity. The tumors consisted of sheets of spindled and pleomorphic cells with frequent intranuclear pseudoinclusions associated with clusters of ectatic vessels surrounded by prominent perivascular hyaline material. Numerous, nonhyalinized vessels were also present, mostly in the peripheral areas of the lesions. Some of these vessels had their walls permeated by numerous small capillaries. Immunostaining for vascular endothelial growth factor (VEGF), a secreted protein that has been implicated in tumor-associated angiogenesis, demonstrated positive staining in both tumoral and endothelial cells. Tumor cells were also reactive to vimentin and CD34. Focal positivity for CD99 and factor XIIIa was also present. Flow cytometry yielded a diploid DNA histogram with S-phase fraction of 7%. Our findings corroborate those from previously reported cases. They further suggest that angiogenesis and the angiogenic factor VEGF may play a role in the development of this peculiar tumor.

Pleomorphic hyalinizing angiectatic tumor of soft parts. A low-grade neoplasm resembling neurilemoma.

Fourteen examples of an unusual mesenchymal tumor characterized by sheets and fascicles of mitotically inactive, hemosiderin-stippled, spindled, and pleomorphic cells, situated around an angiectatic vasculature, are described. The 14 tumours developed in eight women and six men (aged 32-83 years) and ranged in size from 2.3 to 8 cm. Eleven cases presented in the subcutaneous tissues, of which eight were located in the lower extremity. All featured prominent clusters of thin-walled ectatic vessels surrounded by perivascular hyaline material representing a combination of fibrin and collagen. In three cases the perivascular hyalinization was so extensive that it constituted more than half of the total tumor area. The tumor cells were similar to those of malignant fibrous hystiocytoma but differed from them by the presence of prominent intranuclear cytoplasmic inclusions, the extreme scarcity of mitotic figures, and the occasional presence of CD-34 expression. These tumors also shared several features with neurilemomas, such as their unusual vasculature, intranuclear cytoplasmic inclusions, lack of mitotic figures, and abundance of mast cells. They could be distinguished from neurilemomas, however, by the usual presence of infiltrative margins and the absence of S-100 protein. Follow-up information on eight patients (6 months to 25 years) indicated recurrences in four cases, with one of the three patients experiencing numerous recurrences over a 25-year period. No patient has developed metastases, however. We suggest that this tumor is a low-grade sarcoma of uncertain lineage in which the vascular changes are, in part, reflective of its slow growth.