Angiectatic Tumor Research Articles

Pleomorphic hyalinizing angiectatic tumor originating in the nasal cavity: A CARE case report

IntroductionPleomorphic hyalinizing angiectatic tumor (PHAT) is a rare low-grade malignant mesenchymal neoplasm. It commonly occurs in the limbs and trunk, with limited occurrences in the head and neck region. Only five cases of PHAT occurring in the head and neck have been reported in the literature to date. The etiology of PHAT remains unclear. We described a case of nasal cavity PHAT following CARE guidelines. Case summaryA 32-year-old male with PHAT originating in the nasal cavity recurred after two surgeries. Adjuvant radiotherapy was performed after the third surgery. The patient has been regularly followed up for 36months, and no tumor recurrence or metastasis has been observed. DiscussionPHAT is a rare soft tissue tumor known for its local aggressiveness. Because of a high risk of recurrence, extensive resection should be implemented, possibly completed by radiotherapy if resection cannot be complete because of proximity to at-risk structures.

Report of an Unusual Case of Hemosiderotic Fibrohistiocytic Lipomatous Tumor with Systematic Review of Clinicopathological Characteristics and Differential Diagnosis

The spectrum of lipomatous lesions ranges from benign to highly malignant disease. Differentiation between these mesenchymal lesions is important for pathologist as well as clinician to indicate prognosis and choose the most appropriate treatment. Hemosiderotic firohistiocytic lipomatous tumor (HFLT) is a rare subtype of lipomatous tumor. The diagnosis is usually based on clinical, histological, and immunohistochemical (IHC) information. We reported a case of 56 years old man with a painful mass in the dorsal aspect of the thigh showing an unusal clinical presentation but a characteristic histological and IHC features. In this article we have retrospectively reviewed the 67 cases of HFLT reported in literature so far along with one case reported at our institution. The review focuses on clinicopathological and histomorphological characteristics of HFLT and the related entities. The hallmark of HFLT and its related lesions is complex admixture of mature adipose tissue, spindle tumor cells with striking deposition of hemosiderin pigment. Even though HFLT shows marked predeliction for the distal extremeties especially the foot/ ankle with a female preponderance, they can occur at uncommon site such as thigh and also in males as seen in our case. Keywords: Pleomorphic hyalinizing angiectatic tumor, Hemosiderotic fibrolipomatous tumor, Myxoinflammatory Fibroblastic Sarcoma, Hemosiderin, Lipoma

Pleomorphic Hyalinizing Angiectatic Tumor in the Upper Abdomen.

Su, Chaoyun MM1,*; Zhang, Junwei MD2,*; Chen, Xiaoxue MM3; Xu, Yiyao MD2; Lu, Xin MD2 Author Information

Superficial CD34-Positive Fibroblastic Tumor: A Clinicopathologic, Immunohistochemical, and Molecular Study of 59 Cases.

Superficial CD34-positive fibroblastic tumor (SCD34FT) is a rare soft tissue neoplasm that shows overlapping features with PRDM10 -rearranged soft tissue tumor ( PRDM10 -STT). This study characterizes the clinicopathologic, immunohistochemical, and molecular features of SCD34FT in a series of 59 cases. Fluorescence in situ hybridization to assess for PRDM10 rearrangement was performed in 12 tumors. Immunohistochemistry for CADM3 and WT1 was performed; CADM3 was also assessed in histologic mimics. Our cohort of 33 male and 26 female had a median age of 42 (range: 14 to 85) years. Tumors were most commonly located in the lower limb (73%), upper limb (8%), back (7%), and supraclavicular region (3%). The median tumor size was 3.0cm (range: 1.0 to 9.0cm). Clinical follow-up in 32 patients (median duration: 26mo) revealed 2 local recurrences (6%). One patient developed regional lymph node metastases which were completely excised. Microscopically, SCD34FT comprised spindled and pleomorphic cells with glassy cytoplasm and occasional granular cell change. Fluorescence in situ hybridization confirmed PRDM10 rearrangement in 3/8 cases (38%). SCD34FT frequently expressed CADM3 (95%) and WT1 (75%). CADM3 was less diffusely positive in pleomorphic hyalinizing angiectatic tumor (40%), pleomorphic liposarcoma (20%), and undifferentiated pleomorphic sarcoma (10%). We corroborate that SCD34FT is indolent but may rarely metastasize to lymph nodes without adverse outcomes. CADM3 and WT1 may be useful in the distinction from histologic mimics. Since cases of SCD34FT with and without demonstrable PRDM10 rearrangement were clinicopathologically indistinguishable, our study further supports that SCD34FT and PRDM10 -STT likely constitute a single entity.

Pleomorphic hyalinizing angiectatic tumor of the vulva: literature review based on a rare presentation.

ABSTRACTPleomorphic hyalinizing angiectatic tumor (PHAT) of soft tissues is a rare, non-metastatic tumor of unknown etiology and uncertain behavior, which may recur locally. There are few reports on this condition, and due to the rarity of the disease, its lineage has not yet been fully elucidated. The present study aims to report the case of an unusual entity observed for the first time in vulval topography. A female patient, 83 years old, presented with a tumor in the vulvar region that had evolved for approximately 4 months. Magnetic resonance imaging showed an expansive perineal formation of 8.5 × 3.5 cm, and a hemivulvectomy with a flap rotation was performed. The review of the slides revealed a mesenchymal lesion without significant atypia, which was richly vascularized. In the areas of interest, the immunohistochemical (IHC) study demonstrated positivity for CD34, estrogen, and progesterone receptors; it was negative for the other tested markers. Morphological findings associated with the IHC staining panel supported the diagnosis of PHAT. The main morphological features of PHAT are clusters of ectatic vessels of different sizes that show deposits of subendothelial and intraluminal fibrin. Fusiform and pleomorphic cells randomly arranged in leaves or long fascicles intermingle these vessels. It is essential to recognize this entity and consider it among the differential diagnoses of a mesenchymal lesion, given the wide variety of entities that comprise this group of lesions.

Pleomorphic Hyalinizing Angiectatic Tumor (PHAT): Review of the Literature with Case Presentation.

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a very rare entity of soft tissue considered a “neoplasm of uncertain behaviour of connective or other soft tissue” by the World Health Organization (2020). It develops in subcutaneous tissue of the lower extremities, more frequently in the region of the ankle and foot, and rarely as a deep-seated soft tissue mass in locations such as the perineum, buttock, arms, head and neck, and viscera. Although inconsistent cytogenetic data have been reported on PHAT so far, there are potential morphological and genetic overlaps with hemosiderotic fibrolipomatous tumor (HFLT) and myxoinflammatory fibroblastic sarcoma (MIFS). Here we report a case of PHAT at the level of the upper third of the right thigh in a 48-year-old patient and we also focus on the differential diagnoses of these entities and conduct a literature review of reported cases.

A Case of Pleomorphic Hyalinizing Angiectatic Tumor of Neck Mimicking Neurogenic Tumor

A Case of Pleomorphic Hyalinizing Angiectatic Tumor of Neck Mimicking Neurogenic Tumor

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.

PRDM10-rearranged Soft Tissue Tumor: A Clinicopathologic Study of 9 Cases.

Gene fusion transcripts containing PRDM10 were recently identified in low-grade undifferentiated pleomorphic sarcomas (UPS). Here, we describe the morphologic and clinical features of 9 such tumors from 5 men and 4 women (age: 20 to 61 y). Three cases had previously been diagnosed as UPS, 3 as superficial CD34-positive fibroblastic tumor (SCD34FT), 2 as pleomorphic liposarcoma, and 1 as pleomorphic hyalinizing angiectatic tumor. The tumors were located in the superficial and deep soft tissues of the thigh/knee region (4 cases), shoulder (2 cases), foot, trunk, and perineum (1 case each) ranging in size from 1 to 6 cm. All showed poorly defined cellular fascicles of pleomorphic cells within a fibrous stroma with frequent myxoid change and a prominent inflammatory infiltrate. All displayed highly pleomorphic nuclear features, but a low mitotic count. Most tumors were well circumscribed. One of 9 tumors recurred locally, but none metastasized. Immunohistochemically, all were CD34 and showed nuclear positivity for PRDM10; focal positivity for cytokeratins was seen in 5/6 cases. PRDM10 immunoreactivity was evaluated in 50 soft tissue tumors that could mimic PRDM10-rearranged tumors, including 4 cases exhibiting histologic features within the spectrum of SCD34FT. Except for 2/6 pleomorphic liposarcomas and 1/4 myxofibrosarcomas, other tumors did not show nuclear positivity but displayed weak to moderate cytoplasmic immunoreactivity. In conclusion, PRDM10-rearranged soft tissue tumor is characterized by pleomorphic morphology and a low mitotic count. Its morphologic spectrum overlaps with SCD34FT. Clinical features of this small series suggest an indolent behavior, justifying its distinction from UPS and other sarcomas.

A pelvic pleomorphic hyalinizing angiectatic tumor: a rare case report

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a particular rare entity corresponding to soft tissue neoplasm with low malignant potential. The exact etiopathology responsible for the tumor occurrence is not exactly identified. Knowledge of this condition is necessary to avoid misdiagnosis of the lesion and subsequently, to adopt the proper management. Here we report a case of PHAT originating from soft tissue in the pelvic retroperitoneum. According to the litterature, few articles deal with such pathology and such location. This work illustrates features of the tumor, its characteristics, and the adequate management with the histopathological findings.

The t(1;10)(p22;q24) TGFBR3/MGEA5 Translocation in Pleomorphic Hyalinizing Angiectatic Tumor, Myxoinflammatory Fibroblastic Sarcoma, and Hemosiderotic Fibrolipomatous Tumor.

Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts, hemosiderotic fibrolipomatous tumor (HFLT), and myxoinflammatory fibroblastic sarcoma (MIFS) are 3 distinct entities of low-grade spindle cell mesenchymal neoplasm. These tumors have similar clinical presentations and partially overlapping but distinctive pathologic features. A recurrent translocation, t(1;10)(p22;q24), has been detected in a subset of PHAT, HFLT, MIFS, and HFLT/MIFS hybrid cases. Translocation t(1;10)(p22;q24) involves transforming growth factor β-receptor 3 ( TGFBR3) and meningioma-expressed antigen 5 ( MGEA5) genes on chromosomes 1p22 and 10q24, respectively. However, the percentage of translocation in PHAT, HFLT, and MIFS varies significantly among different studies. The relationship among these tumors has been a controversial topic among experts. To discuss the diagnostic and functional significance of translocation t(1;10)(p22;q24) TGFBR3/MGEA5 rearrangement in HFLT, PHAT, and MIFS. PubMed was used for this study. Diagnosis of HFLT, PHAT, and MIFS is challenging because of a lack of unique morphologic, immunophenotypic, molecular, and cytogenetic markers. The recurrent t(1;10)(p22;q24) translocation and/or TGFBR3/MGEA5 rearrangement was reported in 55 patients, with a relatively even distribution among HFLT, PHAT, and MIFS (17 HFLT, 15 MIFS, 13 MIFS/HFLT, and 10 PHAT). This indicates that current morphology-based diagnostic criteria do not identify reliably the subset of soft tissue tumor with t(1;10) translocation. Genetic heterogeneity of these tumors is supported by the recent detection of a mutually exclusive, second recurrent genetic change, t(7;17) TOM1L2-BRAF translocation or BRAF amplification, in a subset of MIFS.

Imaging and histological appearance of pleomorphic hyalinizing angiectatic tumors: A case series and literature review.

Pleomorphic hyalinizing angiectatic tumors (PHATs) are rare mesenchymal soft tissue tumors of uncertain lineage and intermediate malignancy. The present study assesses two cases of PHAT and discusses the histological and immunophenotypical features, as well as the imaging appearance of these tumors on ultrasound, computed tomography (CT), magnetic resonance imaging and positron emission tomography/CT scans. The current study also reviews the literature and discusses the clinical management of these tumors. Wide local excision with tumor free margins is the current recommended treatment for PHAT. Surgical excision may be combined with low-dose radiation to reduce the risk of local recurrence. Patients should be followed up with serial imaging, as PHAT lesions tend to recur locally.

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

Hemosiderotic Fibrolipomatous Tumor, Pleomorphic Hyalinizing Angiectatic Tumor, and Myxoinflammatory Fibroblastic Sarcoma: Related or Not?

Hemosiderotic fibrolipomatous tumor is an unusual, distinctive soft tissue neoplasm with locally recurring potential, which most commonly occurs in the ankle and foot. Morphologic evidence strongly suggests that hemosiderotic fibrolipomatous tumor is related to another rare, locally aggressive tumor of the distal extremities, pleomorphic hyalinizing angiectatic tumor, with areas identical to hemosiderotic fibrolipomatous tumor seen at the periphery in most if not all pleomorphic hyalinizing angiectatic tumor. This morphologic evidence is further supported by molecular genetic data, showing recurrent TGFBR3 and/or MGEA5 rearrangements in both hemosiderotic fibrolipomatous tumor and pleomorphic hyalinizing angiectatic tumor. A possible link between hemosiderotic fibrolipomatous tumor and yet another low-grade sarcoma of the distal extremities, myxoinflammatory fibroblastic sarcoma, has also been suggested based on the occurrence of unusual examples of hemosiderotic fibrolipomatous tumor showing progression to myxoid sarcoma, demonstrating some but not all features of myxoinflammatory fibroblastic sarcoma. These "hybrid hemosiderotic fibrolipomatous tumor-myxoinflammatory fibroblastic sarcoma" also commonly show TGFBR3 and/or MGEA5 rearrangements. However, classic myxoinflammatory fibroblastic sarcoma lacks areas resembling hemosiderotic fibrolipomatous tumor, and shows a very low frequency of TGFBR3 and/or MGEA5 rearrangements in prospectively diagnosed cases. This suggests that so-called "hybrid hemosiderotic fibrolipomatous tumor-myxoinflammatory fibroblastic sarcoma" represents a form of malignant progression within hemosiderotic fibrolipomatous tumor, rather than a lesion strictly related to classic myxoinflammatory fibroblastic sarcoma. This article will review the morphologic features, genetic features, and differential diagnosis of these rare neoplasms, and discuss their interrelation, or lack thereof.

Pleomorphic Hyalinizing Angiectatic Tumour: A Rare Case Report and Discussion of Differential Diagnosis

Pleomorphic Hyalinizing Angiectatic Tumour (PHAT) is one of the rare soft tissue tumour which is non-metastasizing. The origin of this tumour is yet uncertain. It occurs in adults as a slow growing subcutaneous mass mimicking clinically and histologically to various benign and malignant soft tissue tumours such as schwannoma, haemangioma and malignant fibrous histiocytoma. The microscopic features of this tumour include clusters of ectatic, fibrin containing, hyalinized blood vessels with pleomorphic and spindle shaped tumour cells showing intranuclear inclusions, stromal haemosiderin pigment and a variable inflammatory infiltrate. Despite marked pleomorphism, the lesion behaves as a low grade neoplasm, with frequent recurrences, but no metastases. The incidence of this tumour is very rare with less than 100 cases being published. Hence, awareness of this entity is must for proper management of the patient and to avoid misdiagnosis of the lesion. We report a case of pleomorphic hyalinizing angiectatic tumour in a 50-year-old man who presented with a slow growing mass in the left calf region since two years.

TGFBR3 and MGEA5 rearrangements are much more common in “hybrid” hemosiderotic fibrolipomatous tumor-myxoinflammatory fibroblastic sarcomas than in classical myxoinflammatory fibroblastic sarcomas: a morphological and fluorescence in situ hybridization study

Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade sarcoma that most often involves the distal extremities of adults. Some MIFSs have been reported to show TGFBR3 and MGEA5 rearrangements. TGFBR3 and MGEA5 rearrangements have also been reported in hemosiderotic fibrolipomatous tumor (HFLT), in pleomorphic hyalinizing angiectatic tumor (PHAT), and in rare tumors allegedly showing features of both HFLT and MIFS (hybrid HFLT-MIFS). These findings have led to speculation that HFLT, MIFS, PHAT, and hybrid HFLT-MIFS are closely related; however, areas resembling HFLTs are only very rarely encountered in previous series of MIFSs. We studied classic examples of these tumors with the goal of clarifying the relationship between MIFS and HFLT-MIFS. Cases of MIFS (n=31), hybrid HFLT-MIFS (n=8), PHAT (n=2), HFLT (n=1), and undifferentiated pleomorphic sarcoma (n=4) were retrieved from our archives, and the diagnoses were verified by 5 soft tissue pathologists. Using previously validated break-apart fluorescence in situ hybridization probes, we analyzed for TGFBR3 and MGEA5 rearrangements. Only 2 of 31 MIFSs harbored MGEA5 rearrangements; all lacked TGFBR3 rearrangements. Six of 8 hybrid HFLT-MIFSs harbored rearrangements of TGFBR3 and/or MGEA5. Both PHATs were positive for rearrangements of TGFBR3 and/or MGEA5. The HFLT was positive for rearrangements of both TGFBR3 and MGEA5. All undifferentiated pleomorphic sarcomas with focal myxoid change were negative. We conclude that (1) TGFBR3 and/or MGEA5 rearrangements are much more common in hybrid HFLT-MIFSs than in classic MIFSs, (2) HFLTs and MIFSs may be unrelated lesions, and (3) hybrid HFLT-MIFSs most likely represent HFLTs with sarcomatous progression, rather than tumors strictly related to classic MIFSs.

Pleomorphic hyalinising angiectatic tumour: A case report

Pleomorphic hyalinising angiectatic tumour: A case report

Pleomorphic hyalinizing angiectatic tumor revisited: all tumors manifest typical morphologic features of myxoinflammatory fibroblastic sarcoma, further suggesting 2 morphologic variants of a single entity.

We describe 9 cases of pleomorphic hyalinizing angiectatic tumor (PHAT). Recently described TGFBR3 and MGEA5 gene rearrangements in these tumors have confirmed the long-hypothesized link between PHAT and another soft tissue entity, the myxoinflammatory fibroblastic sarcoma (MIFS). Myxoinflammatory fibroblastic sarcoma and PHAT share the same translocation and in addition have a very similar clinical presentation. However, to our best knowledge, no study has ever addressed the striking morphologic similarities between MIFS and PHAT. Our findings based on histological criteria suggest that most, if not all, tumors diagnosed as PHAT might, in fact, represent examples of MIFS that, in addition to a conventional MIFS morphology, manifest aberrant angiectatic hyalinized vessels.

Large pleomorphic hyalinizing angiectatic tumor of the forearm: A multidisciplinary perspective

Large pleomorphic hyalinizing angiectatic tumor of the forearm: A multidisciplinary perspective