scholarly journals Well-Differentiated Liposarcoma of the Hypopharynx Exhibiting Myxoid Liposarcoma-like Morphology with MDM2 and DDIT3 Co-Amplification

2021 ◽  
Author(s):  
Khaled A. Murshed ◽  
Hayan Abo Samra ◽  
Adham Ammar
Keyword(s):  
Spermatic Cord ◽  
Gene Rearrangement ◽  
Rare Event ◽  
Soft Tissue Sarcomas ◽  
Myxoid Liposarcoma ◽  
Posterior Wall ◽  
Myxoid Stroma ◽  
Spindle Cells ◽  
Well Differentiated

AbstractWell-differentiated liposarcoma (WDL) is one of the most common soft tissue sarcomas in adults. It has a predilection for middle-aged males and arises in deep-seated locations such as retroperitoneum, mediastinum, and spermatic cord. Its occurrence in young individuals at the hypopharyngeal region is an exceedingly rare event. Myxoid liposarcoma (ML)-like changes can seldom occur in some cases of WDL, which makes the diagnosis of WDL more challenging. Amplification of DDIT3 gene in a subset of cases of WDL has shown to be associated with such unique morphology. Herein, we present a case of a 36-year-old gentleman who presented with difficulty in breathing and swallowing for 3 months duration. CT scan of the neck revealed a lesion along the posterior wall of the hypopharynx measuring 3.5 cm. Histopathologic examination revealed a tumor composed of lobules of oval to spindle cells in a prominent myxoid stroma with delicate chicken-wire vasculature. In the vicinity, there were lobules composed of variably sized adipocytes separated by thick fibrous septa that contains atypical hyperchromatic spindle cells. By immunohistochemistry, the tumor cells in both components were immunoreactive for CDK4, but negative for MDM2. Fluorescence in-situ hybridization (FISH) confirmed the presence of MDM2 gene amplification. There was no evidence of FUS-DDIT3 gene rearrangement, however, DDIT3 gene was also amplified. The diagnosis of well-differentiated liposarcoma with prominent myxoid stroma was rendered. This is the first documentation of WDL with ML-like morphology harboring co-amplification of MDM2 and DDIT3 in the hypopharynx.

Benign Fibromyxoid Lesion of the Breast: A Distinct Entity From Benign Spindle Cell Tumors of the Mammary Stroma?

2018 ◽  
Vol 26 (6) ◽  
pp. 488-493 ◽  
Author(s):  
Christopher J. Schwartz ◽  
Cynthia A. Schandl ◽  
Jennifer Morse ◽  
Jonathan Ralston ◽  
Amy Rapkiewicz ◽  
...  
Keyword(s):  
Fluorescent In Situ Hybridization ◽  
Gene Rearrangement ◽  
Spindle Cell ◽  
Smooth Muscle Actin ◽  
Distinct Entity ◽  
Myxoid Stroma ◽  
Spindle Cells ◽  
Spindle Cell Proliferation ◽  
Rb Gene

Myxoid lesions of the breast can be diagnostically challenging entities. We report 4 cases of CD34+ fibromyxoid lesion that have been previously diagnosed as “benign myxoid lesion,” “nodular mucinosis,” or “mammary myofibroblastoma, myxoid type” on the basis of CD34-positivity. The lesions were microscopically well circumscribed and composed of a paucicellular spindle cell proliferation in a background of myxoid stroma. No epithelial component was identified. The spindle cells showed immunohistochemical reactivity for CD34 and smooth muscle actin. Based on morphologic and immunohistochemical similarities between these cases and myxoid myofibroblastoma, we compared 4 myxoid lesions with cases of typical myofibroblastoma, utilizing retinoblastoma (Rb) antibody and fluorescent in situ hybridization for 13q14 gene rearrangement (encoding the Rb gene). The myxoid lesions showed retention of Rb protein by immunohistochemistry, whereas Rb expression was lost in cases of myofibroblastoma. We identified loss of 13q14 in 3 of 4 cases of myofibroblastoma. Notably, 13q14 gene rearrangement was not observed in any of the myxoid lesions. Our data show that there is at least a subset of CD34+ fibromyxoid lesions that, despite overlapping morphologic and immunohistochemical phenotype and proposed common histogenesis with myofibroblastomas, is genetically distinct from the latter based on Rb analysis.

Rechallenge with trabectedin in patients with locally advanced or metastatic soft tissue sarcoma following drug holiday: The experience of the French Sarcoma Group (FSG).

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 10062-10062 ◽  
Author(s):  
Esma Saada ◽  
Chahineze Rahal ◽  
Isabelle Ray Coquard ◽  
Antoine Italiano ◽  
Christine Chevreau ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Objective Response ◽  
Locally Advanced ◽  
Soft Tissue Sarcomas ◽  
Myxoid Liposarcoma ◽  
Median Number ◽  
Drug Holiday ◽  
Who Grade ◽  
Well Differentiated ◽  
Number Of Cycles

10062 Background: Trabectedin (T) is a marine-derived alkaloid used to treat advanced soft tissue sarcomas (STS) after ifosfamide and/or anthracyclins failure. Since then, the FSG evaluated the clinical benefit in re-administrating T after an initial hold, either medically indicated or upon patient’s request. Methods: Following an online request, clinical and histopathological data were collected from six centers of the FSG who declared to have rechallenged patients. Baseline data were collected and analyze will be used. Results: From 1999 to 2011, 49 pts with T drug holiday have been identified (26 male/ 23 female), with a median age of 50 y [23-75]. Most frequent histotypes were: myxoid liposarcoma (18, 36.7%), leiomyosarcomas (13, 26.5 %) and well-differentiated/dedifferentiated liposarcoma (9, 18%). WHO grade were 1 in 14 (29%), 2 in 19 (39%) and 3 in 5 (10%) pts respectively. Patients who had a maximum of 2, 3 or 4 therapeutic sequences (TS) with T (drug-holiday and rechallenge) were 41/49 ,7/49 and 1/49 respectively. Median number of cycles for 1, 2, 3 and 4 TS were 7 [3-21], 6 [2-30], 6 [2-9] and 6. Median total number of cycles was 15 [6-43]. Median duration of drug-holiday for 1, 2 and 3 TS were 11 [3-91], 7 [2-29] and 4 months [1-5]. Grade 3-4 toxicities incidence decreased with the number of TS (occurred in 36%, 29%, 14% and 0% of pts with 1, 2, 3 and 4 TS) as well as mean T dose per cycle (1.3 mg/m², 1.2 mg/m², 1.1 mg/m² and 1.1 mg/m² for TS 1, 2, 3, 4). Efficacy decreased with number of TS (Number of CR/PR/SD/PD were 1 (2%)/15 (31%)/33 (67%)/0 for TS1; 0/4 (8%)/29 (59%)/16 (3%) for TS2; 0/1 (14%)/2 (29%)/4 (57%) for TS3 and 0/0/0/1(100%) for TS4). Median overall survival was 5.0 y [2.7-7.3] since T introduction, and 1.5 y [0.1-4.8], 0.8 y [0.5-1.3] and 0.6 y following 2nd, 3rd and 4th T reintroduction respectively. Objective response after TS2 were seen in 4 cases of grade 1 sarcomas. Conclusions: Due to the lack of cumulative toxicities over time with T, its rechallenging in responding patients to T (no progression under T) have to be considered in advanced STS.

scholarly journals Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma with Myxoid Stroma in a Hereditary Retinoblastoma Survivor

2019 ◽  
Vol 6 (2) ◽  
pp. 79-86
Author(s):  
Travis Peck ◽  
Kalla A. Gervasio ◽  
Paul J.L. Zhang ◽  
Carol L. Shields ◽  
Sara E. Lally ◽  
...  
Keyword(s):  
External Beam Radiotherapy ◽  
Molecular Genetic ◽  
Myxoid Liposarcoma ◽  
Atypical Lipomatous Tumor ◽  
Lipomatous Tumor ◽  
Myxoid Stroma ◽  
Molecular Genetic Data ◽  
Mesenchymal Neoplasm ◽  
Myxoid Liposarcomas ◽  
Well Differentiated

Atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDL) is an indolent, locally aggressive mesenchymal neoplasm, most often confined to the lower extremities and retroperitoneum and rarely identified in the orbit. Diagnosis of ALT/WDL can be challenging due to its frequent morphologic overlap with benign adipose lesions and other more aggressive liposarcoma subtypes, including myxoid liposarcoma. We describe a 26-year-old female with a history of hereditary retinoblastoma and external-beam radiotherapy to the orbit, who developed orbital liposarcoma. Although initial morphologic assessment raised the consideration of myxoid liposarcoma, subsequent fluorescein in situ hybridization studies demonstrated MDM2 and DDIT3 coamplification without DDIT3 rearrangement, supporting the diagnosis of ALT/WDL with myxoid stroma. The literature review of previously reported orbital myxoid liposarcomas revealed a morphologic overlap of documented tumors with ALT/WDL, dedifferentiated liposarcoma, and pleomorphic liposarcoma with myxoid stroma as well as an absence of immunohistochemical and molecular genetic data supportive of the diagnosis of myxoid liposarcoma. This case emphasizes the potential overlap of ALT/WDL with myxoid liposarcoma and the increasing importance of molecular genetic studies in the diagnosis, prognosis, and management of orbital liposarcoma.

scholarly journals Giant Spermatic Cord Liposarcoma

2014 ◽  
Vol 99 (4) ◽  
pp. 407-409
Author(s):  
Ugo Grossi ◽  
Antonio Crucitti ◽  
Francesco Pierconti
Keyword(s):  
Knee Joint ◽  
Spermatic Cord ◽  
Correct Diagnosis ◽  
Rare Tumor ◽  
Scrotal Mass ◽  
Myxoid Stroma ◽  
Inguinal Hernias ◽  
Well Differentiated ◽  
Myxoid Sarcoma

Abstract We report a case of giant spermatic cord liposarcoma (SCL) in an 81-year-old patient, presenting with a huge scrotal mass that reached up to the knee joint. SCL is a rare tumor, and about 200 cases have been reported in the literature so far. Although 20% of liposarcomas arise in the retroperitoneum, only 0.1% present as incidental inguinal hernias. The occasional presence of myxoid stroma in well-differentiated liposarcomas can lead to confusion with myxoid sarcoma subtypes. Correct diagnosis is critical and reflects remarkable differences in behavior and therapeutic choices.

A Case Report of Dendritic Fibromyxolipoma in the Back of Pediatric Patient

2020 ◽  
Vol 232 (06) ◽  
pp. 328-330
Author(s):  
Yanling Jin ◽  
Lizhi Zhang
Keyword(s):  
Differential Diagnosis ◽  
Pediatric Patient ◽  
Immunohistochemical Staining ◽  
Myxoid Liposarcoma ◽  
Stellate Cells ◽  
The Elderly ◽  
Myxoid Stroma ◽  
Spindle Cells ◽  
Pathologic Features ◽  
Medical University

Abstract Objective To investigate the pathologic features and differential diagnosis of a pediatric dendritic fibromyxolipoma (DFML). Methods We collected one case of dendritic fibromyxolipoma in the back of a pediatric patient (6 years old, female) diagnosed at the First Affiliated Hospital of Dalian Medical University. The case of DFML was studied by H&E and MaxVision immunohistochemical staining. The clinical and pathological features were analyzed with review of the literatures Results The tumor was round or ovoid in shape and appeared in the surface of back. Microscopically, the tumor was mainly consisted of stellate to spindle cells, rope-like collagen fibers and varying amount of mature lipocytes embedded in myxoid stroma. Immunohistochemical staining showed that spindle cells and stellate cells were positive for vimentin, CD34 and BCL-2. Conclusion DFML is a rare special variant of lipoma. It is benign, and it seems to be misdiagnosed as myxoid liposarcoma prior to the admission to our hospital. The pathological histomorphology and immunohistochemistry phenotypes are helpful to the diagnosis and differential diagnosis. Given that DFML mainly occurs in the elderly, this case is rare and worthy to be reported since it occurs in children.

scholarly journals Cutaneous squamous cell carcinoma arising in hidradenitis suppurativa: A case report

2019 ◽  
Vol 7 ◽  
pp. 2050313X1984735 ◽  
Author(s):  
Catherine F Roy ◽  
Simon F Roy ◽  
Feras M Ghazawi ◽  
Erica Patocskai ◽  
Annie Bélisle ◽  
...  
Keyword(s):  
Squamous Cell Carcinoma ◽  
Cell Carcinoma ◽  
Squamous Cell ◽  
Hidradenitis Suppurativa ◽  
Rare Complication ◽  
Excisional Biopsy ◽  
Necrosis Factor Alpha ◽  
Spindle Cells ◽  
Poorly Differentiated ◽  
Well Differentiated

We present a case of a 64-year-old man who presented with a rapidly growing tumor in the left buttock and intergluteal cleft area, which was affected by hidradenitis suppurativa. The patient was on tumor necrosis factor-alpha inhibitors for hidradenitis suppurativa for 2 years prior to the development of the mass. Initial biopsy of the mass showed a well-differentiated squamous cell carcinoma with spindle cells and positive epithelial immunomarkers. Subsequent excisional biopsy of the tumor showed an infiltrating poorly differentiated squamous cell carcinoma composed of islands of atypical sarcomatoid spindle cells. Squamous cell carcinoma arising in hidradenitis suppurativa is a rare complication which may occur secondary to chronic inflammation and epidermal hyperproliferation in hidradenitis suppurativa–affected areas.

scholarly journals Carbon ion radiotherapy for recurrent calf myxoid liposarcoma: a case report

2021 ◽  
Vol 49 (4) ◽  
pp. 030006052110097
Author(s):  
Xiaojun Li ◽  
Yanshan Zhang ◽  
Yancheng Ye ◽  
Ying Qi ◽  
Chunlan Feng ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Myxoid Liposarcoma ◽  
Ion Beams ◽  
Carbon Ion Radiotherapy ◽  
Irradiation Damage ◽  
Radiation Dermatitis ◽  
Complete Disappearance ◽  
Carbon Ion ◽  
The Right

Liposarcoma (LPS) is the most common soft tissue sarcoma. Myxoid LPS (MLPS) is the second most common subtype of LPS and accounts for 25% to 50% of all LPSs. Like most other soft tissue sarcomas, the mainstay of treatment for LPS is inevitably surgery. Multidisciplinary approaches, including surgery, chemotherapy, and radiotherapy, have been successful in the treatment of LPS during the last three decades. Even so, recurrence of LPS remains challenging. Carbon ion beams produce increased energy deposition at the end of their range to form a Bragg peak while minimizing irradiation damage to surrounding tissues, which facilitates more precise dosage and localization than that achieved with photon beams. Furthermore, carbon ion beams have high relative biologic effectiveness. We herein describe a patient who developed recurrent MLPS in the right calf after two surgeries and underwent carbon ion radiotherapy (CIRT), achieving complete disappearance of the tumor. The patient developed Grade 1 radiation dermatitis 30 days after CIRT, but no other acute toxicities were observed. The tumor had completely disappeared by 120 days after CIRT, and the patient remained disease-free for 27 months after CIRT. The CARE guidelines were followed in the reporting of this case.

Mammary-type Myofibroblastoma with Leiomyomatous Differentiation: A Rare Variant with Potential Pitfalls

2021 ◽  
pp. 106689692110313
Author(s):  
Alexander M. Strait ◽  
Julia A. Bridge ◽  
Anthony J. Iafrate ◽  
Marilyn M. Li ◽  
Feng Xu ◽  
...  
Keyword(s):  
Adipose Tissue ◽  
Transcriptome Sequencing ◽  
Smooth Muscle Actin ◽  
The Body ◽  
Muscle Actin ◽  
Mature Adipose Tissue ◽  
Spindle Cells ◽  
Whole Transcriptome Sequencing ◽  
Whole Transcriptome

Myofibroblastoma is a rare, benign stromal tumor with a diverse morphologic spectrum. Mammary-type myofibroblastoma (MTMF) is the extra-mammary counterpart of this neoplasm and its occurrence throughout the body has become increasingly recognized. Similar morphologic variations of MTMF have now been described which mirror those seen in the breast. We describe a case of intra-abdominal MTMF composed of short fascicles of eosinophilic spindle cells admixed with mature adipose tissue. The spindle cells stained diffusely positive for CD34, desmin, smooth muscle actin, and h-caldesmon by immunohistochemistry. Concurrent loss of RB1 (13q14) and 13q34 loci were confirmed by fluorescence in situ hybridization whereas anchored multiplex PCR and whole transcriptome sequencing did not reveal any pathognomonic fusions suggesting an alternative diagnosis. To the best of our knowledge this is the first documented case of leiomyomatous variant of MTMF.

scholarly journals HHV8-Positive Castleman Disease and In Situ Mantle Cell Neoplasia within Dermatopathic Lymphadenitis, in Longstanding Psoriasis

Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1150
Author(s):  
Magda Zanelli ◽  
Luca Stingeni ◽  
Maurizio Zizzo ◽  
Giovanni Martino ◽  
Francesca Sanguedolce ◽  
...  
Keyword(s):  
Mantle Cell Lymphoma ◽  
Cell Lymphoma ◽  
Rare Event ◽  
Castleman Disease ◽  
Axillary Lymph ◽  
Tnf Inhibitor ◽  
Dermatopathic Lymphadenitis ◽  
Multicentric Castleman Disease ◽  
Mantle Cell

A 73-year-old man presented with multiple lymphadenopathy. He had a 20-year history of palmoplantar psoriasis evolved to a diffuse erythrodermic picture in the last two years. Topic and systemic medications including prednisolone, acitretin, anti-IL17 (ixekizumab), TNF inhibitor (adalimumab), anti-IL23 (guselkumab), methotrexate, cyclosporine, and phosphodiesterase 4 inhibitor (apremilast) were ineffective. Repeated skin biopsies excluded mycosis fungoides, confirming psoriasis; molecular analysis of T-cell receptor genes ruled out clonality. The axillary lymph node histology documented a dermatopathic lymphadenitis, often associated with chronic cutaneous inflammatory diseases. At an accurate morphological evaluation, features of HHV8-positive multicentric Castleman disease were observed. Moreover, in a few follicles, in situ mantle cell neoplasia was identified. The translocation t(11;14)(q13;q32), characteristic of mantle cell lymphoma, and the monoclonal IGH gene rearrangement were present. HHV8 DNA was identified on plasma sample. Multicentric Castleman disease in psoriatic patients is a rare event and it might be favored by the immunomodulatory treatment in longstanding psoriasis. Multicentric Castleman disease patients are predisposed to developing simultaneous or subsequent lymphoma. In situ mantle cell neoplasia often behaves indolently, although it may progress to overt mantle cell lymphoma. Rituximab achieved a good control of psoriasis. Unfortunately, the patient developed Staphylococcus aureus sepsis for which he is currently on antibiotic therapy.

Testicular Carcinoma in Situ Associated with Rhabdomyosarcoma of the Spermatic Cord

1989 ◽  
Vol 142 (2 Part 1) ◽  
pp. 358-360 ◽  
Author(s):  
Manuel Nistal ◽  
Carmen Fachal ◽  
Ricardo Paniagua
Keyword(s):  
Spermatic Cord ◽  
Carcinoma In Situ ◽  
Testicular Carcinoma
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