Cytologic and Histologic Features of Pleomorphic Undifferentiated Sarcoma Arising in a Hybrid Hemosiderotic Fibrolipomatous Tumor and Pleomorphic Hyalinizing Angiectatic Tumor: Report of an Unusual Case with a Literature Review

2015 ◽  
Vol 59 (6) ◽  
pp. 493-497 ◽  
Author(s):  
Elizabeth Morency ◽  
William Laskin ◽  
Xiaoqi Lin

Background: Pleomorphic hyalinizing angiectatic tumor (PHAT) and hemosiderotic fibrolipomatous tumor (HFLT) are low-grade neoplasms that share clinicopathologic features and recurring translocation t(1;10)(p22;q24) involving the TGFBR3 and MGEA5 genes. Coexistence of these tumors with a high-grade sarcoma is exceedingly rare and the cytologic features have not been widely described in the literature. Case: A 55-year-old female presented with a soft tissue tumor on the dorsum of the foot. Cytologic smears and corresponding core biopsies were composed of a population of markedly pleomorphic spindle cells seen singly and in loose clusters within a myxofibrous matrix and infiltrating fat, with coarse chromatin, prominent nucleoli, irregular nuclear contours and delicate to vacuolated cytoplasm. Intracytoplasmic hemosiderin granules and rare intranuclear cytoplasmic pseudoinclusions were identified. The histologic features of the excisional biopsy mirrored those of the cytologic preparations, but also demonstrated cellular foci of higher-grade sarcoma composed of markedly pleomorphic tumor cells with large vesicular nuclei and prominent nucleoli, exhibiting a mitotic index of 12 mitotic figures per 10 high-powered fields. Conclusion: While HFLT/PHAT generally can be managed by wide local excision, it is important to be aware of their capacity to harbor higher-grade lesions with metastatic potential which may require more radical surgical excision.

2003 ◽  
Vol 127 (3) ◽  
pp. 297-304 ◽  
Author(s):  
Hsuan-Ying Huang ◽  
Cristina R. Antonescu

Abstract Context.—Smooth muscle tumors (SMTs) of the sinonasal tracts are very rare mesenchymal neoplasms, and the literature includes very limited data correlating histologic parameters with clinical outcome. As the behavior of SMTs is site-dependent, defining applicable criteria to distinguish among low-grade leiomyosarcoma, SMT of uncertain malignant potential (SMTUMP), and cellular leiomyoma is sometimes difficult and arbitrary. Objective.—To correlate the clinicopathologic features of 12 well-differentiated sinonasal SMTs with MIB-1 index and clinical outcomes so as to better classify this group of tumors. Design.—Twelve cases of sporadic well-differentiated SMTs arising from the sinonasal tract were retrieved from both institutions. High-grade leiomyosarcomas were excluded from this analysis. The histologic parameters assessed included circumscription, mucosal ulceration, cellularity, nuclear atypia, mitotic count, necrosis, and destruction of adjacent bony structures. The histologic classification of these tumors was based on the guidelines for SMTs of deep soft tissues, using greater than 4 mitotic figures (MF)/10 high-power fields (HPF) to separate SMTUMP from leiomyosarcoma and the presence of 1 to 4 MF/10 HPF to distinguish between leiomyoma and SMTUMP. Immunostaining of MIB-1 index was performed in 7 cases with available material. Results.—This study showed a 1:1.5 male-female ratio and a mean age of 40 years (range, 20–67 years). The most frequent symptoms were nasal stuffiness and/or epistaxis. The tumors involved nasal cavity in 8 cases (67%), paranasal sinus alone in 2 cases (16.5%), and both nasal cavity and paranasal sinuses in 2 cases (16.5%). The tumors ranged in size from 0.3 to 5.5 cm (mean, 2 cm) and were classified as follows: 7 leiomyomas, 2 SMTUMPs, and 3 low-grade leiomyosarcomas. All 7 leiomyomas, 1 SMTUMP, and 1 leiomyosarcoma originated from vessel walls. Bone involvement was seen in both the SMTUMPs (1 of 2) and leiomyosarcomas (2 of 3). Focal infiltrative growth was observed only in the 3 leiomyosarcomas, 1 of which also showed microscopic coagulative tumor necrosis. Mitotic figures ranged from 0 to 10 (mean, 2.3), with absence of mitosis in all 7 leiomyomas, 1 to 4 MF/10 HPF in the 2 SMTUMPs, and more than 4 MF/10 HPF in the 3 low-grade leiomyosarcomas. The MIB-1 index was low (≤5%) in both leiomyoma and SMTUMP groups, while the leiomyosarcomas showed a higher (≥15%) proliferative index. All 12 patients were treated by surgical excision, and only 1 patient with leiomyosarcoma received postoperative radiation. In all 12 tumors, there was neither local recurrence nor distant metastasis after an average of 93 months of follow-up (range, 4–221 months). Conclusion.—Well-differentiated sinonasal SMTs are rare and occur in adults with a slight female predilection. The lesions confined within the nasal cavity were more common than those involving a single paranasal sinus or both nasal cavity and paranasal sinuses. Despite variations in location, clinical manifestation, histologic features, and MIB-1 index, these tumors appear to follow a favorable course. Complete surgical excision appears to provide adequate treatment for these patients.


2017 ◽  
Vol 5 (2) ◽  
pp. 112
Author(s):  
Gaurav Mahajan ◽  
Aditi Gautam

Pyogenic granuloma is one of the inflammatory hyperplasias seen in the oral cavity. This term is a misnomer because the lesion is unrelated to infection and in reality arises in response to various stimuli such as low- grade local irritation, traumatic injury or hormonal factors. The clinical diagnosis of such lesion can be quite challenging because of its close resemblance to certain malignancies like peripheral giant cell granuloma, kaposi’s sarcoma, non hodgkin’s lymphoma etc. for excisional biopsy, options available are conventional surgical excision, electrocautery or lasers. In this report, we seek to highlight the therapeutic advantages achieved with a soft tissue diode laser in the treatment of pyogenic granuloma.


BMC Urology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Dong In Jo ◽  
Song Hyun Han ◽  
Soon Heum Kim ◽  
Hye Young Kim ◽  
Hong Chung ◽  
...  

Abstract Background Verrucous carcinoma, a rare low-grade well-differentiated squamous cell carcinoma, is known for its favorable biological behavior and lack of metastatic potential. However, aggressive resection is problematic in terms of compromised function and aesthetics. Hence, more conservative treatments are needed. Methods: To identify the up-to-date general biological behavior, diagnosis, and treatment trends, we searched PubMed using the keyword “penile verrucous carcinoma” without restrictions on publication date. Results: Current treatments for penile verrucous carcinoma include wide surgical excision, seldom preventive lymphadenectomy, and conservative chemotherapy without surgery or local excision with safe margins. Despite the advent of partial penectomy to minimally impact function and aesthetics, affected patients experience psychosexual problems. Local excision can be used to save the penile shaft and glans penis without preventive lymphadenectomy or adjuvant therapy and can achieve good clinical prognosis with rare recurrence. Conclusions: To preserve the functional and cosmetic aspects, we recommend local excision, especially for tumors measuring < 3 cm and classified as stage T1 according to the 2016 tumor node metastasis clinical and pathological classification for penile cancer.


2017 ◽  
Vol 26 (1) ◽  
pp. 73-77
Author(s):  
Neha Gupta ◽  
Samuel Kenan ◽  
Leonard B. Kahn

Synovial sarcoma is a high-grade sarcoma commonly affecting young adults. The sites of involvement include soft tissue near joints, lung, pleura, mediastinum, larynx, kidney, and buttocks. Histologic types include monophasic, biphasic, and undifferentiated. We report a unique case of synovial sarcoma with low-grade histologic features mimicking pleomorphic hyalinizing angiectatic tumor (PHAT) with indolent behavior for a period of 10 years. The tumor showed angiectatic blood vessels with fibrinous cuffing, hypocellular and hypercellular spindle cell areas with rare mitoses, and focal atypia in a myxoid background. TLE1 was positive with SYT gene translocation detected on fluorescent in situ hybridization. Cases of myxoinflammatory fibroblastic sarcoma and myxofibrosarcoma have been reported as exhibiting histologic features of PHAT. However, to the best of our knowledge, cases of synovial sarcoma mimicking PHAT have not been reported.


2020 ◽  
Vol 40 (4) ◽  
pp. 284-288 ◽  
Author(s):  
Fabricia H. Loures ◽  
Lissandro G. Conceição ◽  
Renee L. Amorim ◽  
Juliano Nobrega ◽  
Rafael T. Neto ◽  
...  

ABSTRACT: Canine soft tissue sarcomas (STS) comprise a heterogeneous group of malignancies that share similar histopathological features, a low to moderate recurrence rate and low metastatic potential. In human medicine, the expression of estrogen receptors (ER) and progesterone receptors (PR) in sarcomas has been studied to search for prognostic factors and new treatment targets. Similar studies have yet to be conducted in veterinary medicine. The objective of this study was therefore to investigate by immunohistochemistry (IHC) the ER and PR expression in a series of 80 cutaneous and subcutaneous sarcomas in dogs with histopathological features of peripheral nerve sheath tumor (PNST) and perivascular wall tumor (PWT). All cases were positive for PR and negative for ER. Tumors of high malignancy grade (grade III) exhibited higher PR expression than low-grade tumors (grade I). Tumors with mitotic activity greater than 9 mitotic figures/10 high power fields also exhibited higher PR expression. In addition, there was a positive correlation between cell proliferation (Ki67) and PR expression. Therefore, it is possible that progesterone plays a greater role than estrogen in the pathogenesis of these tumors. Future studies should explore the potential for selective progesterone receptor modulators as therapeutic agents in canine STS, as well as evaluating PR expression as a predictor of prognosis.


2021 ◽  
Author(s):  
Abbas Agaimy ◽  
Norbert Meidenbauer ◽  
William R. Sukov ◽  
Robert Stoehr ◽  
Michael Vieth ◽  
...  

AbstractPleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts and hemosiderotic fibrolipomatous tumor (HFLT) are two rare low-grade locally recurring neoplasms with predilection for the foot/ankle. Recent studies support a close link between the two entities, and origin of PHAT from HFLT and occurrence of hybrid HFLT/PHAT have been documented. Both lesions often harbor TGFBR3 or MGEA5 rearrangements. Rare sarcomas originating from HFLT/PHAT have been reported, typically resembling myxofibrosarcoma or myxoinflammatory fibroblastic sarcoma. We describe a novel SMARCA4-deficient undifferentiated sarcoma with rhabdoid features originating from hybrid HFLT/PHAT in the foot of a 54-year-old male. The tumor pursued a highly aggressive course with rapid regrowth after resection and multiple metastases resulting in patient’s death within 5 months, despite systemic chemotherapy. Immunohistochemistry revealed SMARCA4 loss in the undifferentiated sarcoma, but not in the HFLT/PHAT. Molecular testing confirmed TGFBR3/MGEA5 rearrangements. This report expands the phenotypes of sarcomas developing from pre-existing PHAT/HFLT.


2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Stephanie M. Tillit ◽  
Siva S. R. Iyer ◽  
Eric J. Grieser ◽  
John T. LiVecchi

Primary cutaneous mucinous carcinoma (PCMC) is a rare, low-grade malignant neoplasm of the sweat gland, whose history has been controversial regarding eccrine versus apocrine origin. This case report describes a 53-year-old male who presented to the University of Florida, Gainesville, ophthalmology clinic and was referred to the oculoplastics service with a painless, subcentimeter mass on the lateral right upper eyelid including the canthus, consistent with recurrent primary cutaneous mucinous carcinoma of the eyelid. Four years prior, the patient had undergone excisional biopsy of the lesion in a clinic, which revealed residual tumor, but the patient deferred further surgery at the time. The patient underwent surgical excision of the mass with reconstruction without operative complications and with negative surgical margins. PCMC is difficult to diagnose clinically due to its rarity and requires a histopathological examination for confirmation of the diagnosis. This report presents the first case in the literature of primary cutaneous mucinous carcinoma in a patient with human immunodeficiency virus (HIV). With this case report, we aim to raise awareness of primary cutaneous mucinous carcinoma as a potential part of the differential diagnosis for malignant eyelid lesions, including those present in patients with HIV.


2021 ◽  
Vol 79 (3) ◽  
pp. 265-268
Author(s):  
Isabela Guerra ◽  
Hisabella Lorena Porto Simões ◽  
Marcella Amaral Horta Barbosa Vieira ◽  
Maria Christina Marques Nogueira Castañon

Carcinoma cuniculatum is a rare variant of low-grade and well-differentiated squamous cell carcinoma. It is a locally invasive tumor, although it has low metastatic potential. It was originally described in the plantar region but may exceptionally appear in other locations. It predominates in middle-aged men and presents clinically as an exophytic, solitary, painful tumor, with insidious growth. Histologically, this tumor simulates a variety of benign dermatoses and may require several biopsies for the correct diagnosis. It is often misdiagnosed as a viral wart, due to the clinical similarity and the indolent course. The treatment of choice is surgical excision due to the high risk of recurrence and locally aggressive behavior. Amputation can be performed in cases of deep tissue invasion. We present a case of carcinoma cuniculatum in which the late diagnosis favored the invasion of the underlying bone, resulting in amputation of the affected finger.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Jessica Ellis ◽  
Keziah Austin ◽  
Sarah Emerson

Abstract Background/Aims  A 49-year-old female of Nepalese heritage was referred with right-sided headache, scalp tenderness, and a painful swelling overlying the right temple. She denied any visual or claudicant symptoms but felt systemically unwell with a fever. There were no symptoms suggestive of an inflammatory arthritis, underlying connective tissue disease or vasculitis. She was normally fit and well with no past medical history. She did not take any regular medications and denied using over the counter or illicit drugs or recent travel. On review she had a low grade fever. There was a large tender, erythematous swelling overlying the right temple. Bilaterally the temporal arteries were palpable and pulsatile. Peripheral pulses were normal with no bruits. There was no evidence of shingles (HSV) or local infection. Full systemic examination revealed no other abnormalities. Laboratory tests showed: PV 2.56, CRP 101, total white cell count 14.38 (eosinophils 0.4), albumin 33, Hb 115. Urine dip was normal. Renal function, liver function and immunoglobulins were normal. ANCA was negative. Hypoechogenicity surrounding the right frontal branch of the right temporal artery was seen on ultrasound. There were no discrete masses suggestive of cysts, abscess or tumours. Temporal artery biopsy confirmed the presence of vasculitis; histology demonstrated transmural lymphohistiocytic inflammation, disruption of the elastic lamina and intimal proliferation. Prednisolone was started at 40mg daily. Four weeks after initially presenting she was asymptomatic and her inflammatory markers had normalised. Methods  The case is discussed below. Results  Temporal arteritis, or GCA, is primarily a disease of older adults; with age 50 often used as an inclusion criteria, and is more common in Caucasian populations. Limited reports exist of GCA in younger cohorts, but these are rare. An important differential in younger patients, such as ours, is juvenile temporal arteritis. This rare localised vasculitis affects almost exclusively the temporal artery. It is typically a disease of young males, who present with non-tender temporal swelling. Systemic symptoms are unusual and inflammatory markers are normal. Clinical or laboratory evidence of organ involvement, peripheral eosinophilia or fibrinoid necrosis on histology should prompt consideration of an AAV or PAN. Incidence of GCA increases in correlation with Northern latitude, with highest rates reported in Scandinavian and North American populations. GCA is rare in Asian populations. Higher diagnostic rates in countries where physicians have increased awareness of GCA proposed as an explanation for this difference; however differences in incidence are still observed between Asian and Caucasian populations presenting to the same healthcare providers. Conclusion  GCA is an uncommon diagnosis in younger and non-Caucasian patients. Thorough investigation through ultrasound and biopsy helped increase our diagnostic confidence in this unusual case. Rheumatologists must be alert to atypical presentations in order to deliver prompt and potentially sight-saving treatment. Disclosure  J. Ellis: None. K. Austin: None. S. Emerson: None.


2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Abelardo Loya-Solis ◽  
Karla Judith González-Colunga ◽  
Cynthia M. Pérez-Rodríguez ◽  
Natalie Sofía Ramírez-Ochoa ◽  
Luis Ceceñas-Falcón ◽  
...  

Ameloblastic fibrosarcoma is an uncommon odontogenic tumor composed of a benign epithelial component and a malignant ectomesenchymal component most frequently seen in the third and fourth decades of life. It mainly presents as a painful maxillary or mandibular swelling. Radiographs show a radiolucent mass with ill-defined borders. Radical surgical excision and long-term follow-up are the suggested treatment. We report the case of a 22-year-old female with a 2-month history of an asymptomatic swelling in her left mandible. Examination revealed an exophytic growth measuring3×3 cm extending from the mandibular left first premolar to the second molar. The patient underwent a left hemimandibular resection. Histopathological examination revealed a biphasic tumor composed of inconspicuous islands of benign odontogenic epithelium and an abundant malignant mesenchymal component with marked cellularity, nuclear pleomorphism, hyperchromatism, and moderate mitotic figures with clear margins; one year after the surgical procedure, the patient is clinically and radiologically disease-free.


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