Clinical and histopathological evaluation of cutaneous angiofibromas

Nasimi, Maryam; Kamyab, Kambiz; Moradi, Arezou; Dasdar, Shayan; Kianfar, Nika

doi:10.1111/cup.14060

Cited by 3 publications

(3 citation statements)

References 16 publications

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“…[12] Microscopically, it is shown as short spindle cells with unequal distributed and diffused thin-walled lobulated blood vessels in a mucous or glioidal background, on immunophenotyping, it expresses vimentin, CD31, CD34, in addition to myogenic antibodies. [13] Schwannoma is a benign tumor originating from Schwan cells that encases the peripheral nerves with intact It may occasionally arise in peripheral nerves of the limbs. Schwannoma is more prone to focal bleeding, necrosis, and cystic degeneration, and the nearby nerves at both ends show a specific manifestation of "rat tail sign" on ultrasonography for identification.…”

Section: Discussionmentioning

confidence: 99%

“…[ 12 ] Microscopically, it is shown as short spindle cells with unequal distributed and diffused thin-walled lobulated blood vessels in a mucous or glioidal background, on immunophenotyping, it expresses vimentin, CD31, CD34, in addition to myogenic antibodies. [ 13 ] Schwannoma is a benign tumor originating from Schwan cells that encases the peripheral nerves with an intact envelope. It may occasionally arise in peripheral nerves of the limbs.…”

Section: Discussionmentioning

confidence: 99%

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Inflammatory myofibroblastic tumor of the upper arm: A case report

Yuan,

Fan,

2023

Medicine

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Rationale: Inflammatory myofibroblastic tumor (IMT) is an uncommon benign myofibroblastic tumor that usually occurs in the lung, mediastinum, abdomen and vulvovaginal region. IMT of the upper arm is exceedingly rare with unknown etiology. Pathology plays a major role in the diagnosis of IMT, and radiological characteristics of the condition are crucial for differential diagnosis. Patient concerns: A 62-year-old woman was admitted to our hospital for a complaint of a mass in her left upper limb with progressive numbness in the extremity. Ultrasound examination of the brachial artery (BA) revealed a hypoechoic mass with well-defined borders and a substantial blood flow, and the mass was also shown to be greatly enhanced on computed tomography (CT) and magnetic resonance imaging (MRI). Diagnosis: The subsequent histopathological and immunohistochemical studies led to the diagnosis of IMT. Intervention: The patient was referred for surgery. The soft tissue tumor resection, left median nerve release operation, brachial artery vascular grafting, and arterial anastomosis were performed. Outcome: Favorable outcome was observed. The patient recovered well from the procedure and did not experience any further complications or tumor recurrence. Lessons: In this report, we describe a case of IMT of the upper arm with BA involvement. The case expands the differential diagnosis of limb neoplasm and broadens the understanding of its ultrasonic and radiological imaging features. It also serves as a further example of an uncommon region distinct from conventional IMT. Further studies on the etiology and therapeutic strategies are needed.

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Section: Discussionmentioning

confidence: 99%

“…[ 12 ] Microscopically, it is shown as short spindle cells with unequal distributed and diffused thin-walled lobulated blood vessels in a mucous or glioidal background, on immunophenotyping, it expresses vimentin, CD31, CD34, in addition to myogenic antibodies. [ 13 ] Schwannoma is a benign tumor originating from Schwan cells that encases the peripheral nerves with an intact envelope. It may occasionally arise in peripheral nerves of the limbs.…”

Section: Discussionmentioning

confidence: 99%

Inflammatory myofibroblastic tumor of the upper arm: A case report

Yuan,

Fan,

2023

Medicine

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“…Angiofibromas are the most frequent skin manifestation associated with MEN1 syndrome, according to most studies [ 48 , 69 , 73 ]. They are benign cutaneous vascular tumors that contain thin-walled blood vessels, stellate and spindled cells, and collagen bundles, and they present as pink or red shiny surfaced papules [ 74 , 75 ]. Apart from MEN1, multiple facial angiofibromas can be found in Birt-Hogg-Dubé syndrome and tuberous sclerosis as well [ 75 ].…”

Section: Men1 and Skin Tumor Profilementioning

confidence: 99%

Approach of Multiple Endocrine Neoplasia Type 1 (MEN1) Syndrome–Related Skin Tumors

et al. 2022

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Non-endocrine findings in patients with MEN1 (multiple endocrine neoplasia) syndrome also include skin lesions, especially tumor-type lesions. This is a narrative review of the English-language medical literature including original studies concerning MEN1 and dermatological issues (apart from dermatologic features of each endocrine tumor/neuroendocrine neoplasia), identified through a PubMed-based search (based on clinical relevance, with no timeline restriction or concern regarding the level of statistical significance). We identified 27 original studies involving clinical presentation of patients with MEN1 and cutaneous tumors; eight other original studies that also included the genetic background; and four additional original studies were included. The largest cohorts were from studies in Italy (N = 145 individuals), Spain (N = 90), the United States (N = 48 and N = 32), and Japan (N = 28). The age of patients varied from 18 to 76 years, with the majority of individuals in their forties. The most common cutaneous tumors are angiofibromas (AF), collagenomas (CG), and lipomas (L). Other lesions are atypical nevi, basocellular carcinoma, squamous cell carcinoma, acrochordons, papillomatosis confluens et reticularis, gingival papules, and cutaneous T-cell lymphoma of the eyelid. Non-tumor aspects are confetti-like hypopigmentation, café-au-lait macules, and gingival papules. MEN1 gene, respective menin involvement has also been found in melanomas, but the association with MEN1 remains debatable. Typically, cutaneous tumors (AF, CG, and L) are benign and are surgically treated only for cosmetic reasons. Some of them are reported as first presentation. Even though skin lesions are not pathognomonic, recognizing them plays an important role in early identification of MEN1 patients. Whether a subgroup of MEN1 subjects is prone to developing these types of cutaneous lesions and how they influence MEN1 evolution is still an open issue.

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Cutaneous lesions and other non-endocrine manifestations of Multiple Endocrine Neoplasia type 1 syndrome

et al. 2023

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BackgroundMultiple Endocrine Neoplasia type 1 is a rare genetic syndrome mainly caused by mutations of MEN1 gene and characterized by a combination of several endocrine and non-endocrine manifestations. The objective of this study was to describe cutaneous lesions and other non-endocrine manifestations of MEN1 in a cohort of patients with familial (F) and sporadic (S) MEN1, compare the prevalence of these manifestations between the two cohorts, and investigate the correlation with MEN1 mutation status.MethodsWe collected phenotypic and genotypic data of 185 patients with F-MEN1 and S-MEN1 followed from 1997 to 2022. The associations between F-MEN1 and S-MEN1 or MEN1 mutation-positive and mutation-negative patients and non-endocrine manifestations were determined using chi-square or Fisher’s exact tests or multivariate exact logistic regression analyses.ResultsThe prevalence of angiofibromas was significantly higher in F-MEN1 than in S-MEN1 in both the whole (p < 0.001) and index case (p = 0.003) cohorts. The prevalence of lipomas was also significantly higher in F-MEN1 than in S-MEN1 (p = 0.009) and in MEN1 mutation-positive than in MEN1 mutation-negative (p = 0.01) index cases. In the whole cohort, the prevalence of lipomas was significantly higher in MEN1 mutation-positive compared to MEN1 mutation-negative patients (OR = 2.7, p = 0.02) and in F-MEN1 than in S-MEN1 (p = 0.03), only after adjustment for age. No significant differences were observed for the other non-endocrine manifestations between the two cohorts. Hibernoma and collagenoma were each present in one patient (0.5%) and meningioma and neuroblastoma in 2.7% and 0.5%, respectively. Gastric leiomyoma was present in 1.1% of the patients and uterine leiomyoma in 14% of women. Thyroid cancer, breast cancer, lung cancer, basal cell carcinoma, melanoma, and colorectal cancer were present in 4.9%, 2.7%, 1.6%, 1.6%, 2.2%, and 0.5% of the whole series, respectively.ConclusionsWe found a significantly higher prevalence of angiofibromas and lipomas in F-MEN1 compared with S-MEN1 and in MEN1 mutation-positive compared to MEN1 mutation-negative patients. In patients with one major endocrine manifestation of MEN1, the presence of cutaneous lesions might suggest the diagnosis of MEN1 and a possible indication for genetic screening.

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Clinical and histopathological evaluation of cutaneous angiofibromas

Cited by 3 publications

References 16 publications

Inflammatory myofibroblastic tumor of the upper arm: A case report

Inflammatory myofibroblastic tumor of the upper arm: A case report

Approach of Multiple Endocrine Neoplasia Type 1 (MEN1) Syndrome–Related Skin Tumors

Cutaneous lesions and other non-endocrine manifestations of Multiple Endocrine Neoplasia type 1 syndrome

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