scholarly journals Myoepithelioma-like tumor of the vulvar region: a case report in China and review of the literature

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Yan Xu ◽  
Hui Gao ◽  
Jin-Li Gao

Abstract Background Myoepithelioma-like tumor of the vulvar region (MELTVR) is a recently described mesenchymal neoplasm which typically arising in vulvar regions of adult women. Case presentation Here we report a case of a 65-year-old woman who presented with a 6-year history of subcutaneous mass in the vulvar region. The mass had recently increased in size continuously. Histologically, the tumor cells had an epithelioid to spindled shape. Epithelioid tumor cells proliferated singly or in a loosely cohesive manner with myxoid areas, while spindled tumor cells grew in diffuse sheets or storiform arrangements mainly in nonmyxoid areas. Immunohistochemically, the tumor cells were positive for vimentin, epithelial membrane antigen, calponin, and were partially mild to moderate positive for estrogen receptor, but completely negative for S100 protein, glial fibrillary acidic protein, CD34, desmin, SMA and cytokeratin. INI1/SMARCB1 expression was deficient. EWSR1 and FUS genes were intact tested by fluorescence in situ hybridization analysis. Based on these findings, we diagnose this case as MELTVR. The patient remained relapse-free after the lesion was widely excised during 8 months follow-up. Conclusions This disease should be included in the differential diagnostic list of vulvar tumors with epithelioid to spindled morphology. Recognition of its histopathological features and immunohistochemical reactivity will help to understand the tumor better.

2021 ◽  
pp. 106689692199604
Author(s):  
Tess Tomassen ◽  
Cees van de Ven ◽  
Jakob Anninga ◽  
Christian Koelsche ◽  
Laura S. Hiemcke-Jiwa ◽  
...  

Nodular fasciitis is usually a benign lesion genetically characterized by ubiquitin-specific protease 6 ( USP6) rearrangements. We present a case of a 10-year-old boy with a 1.5-week history of a painless mass on the right chest wall, which was excised. A histomorphologically malignant tumor with pronounced pleomorphism, atypical mitotic figures, and a myoid immunophenotype was observed. The methylation profile was consistent with nodular fasciitis and fluorescence in situ hybridization confirmed USP6 rearrangement. Using Archer Fusion Plex (Sarcoma Panel) and RNA sequencing, a collagen, type VI, alpha 2 ( COL6A2) –USP6 gene fusion was subsequently identified. Furthermore, DNA clustering analysis also showed a match with nodular fasciitis. During the follow-up of 22 months, no recurrence or metastasis occurred. In conclusion, we describe a clinically benign, histomorphologically malignant mesenchymal neoplasm with a myoid immunophenotype, and a genetic and epigenetic profile consistent with nodular fasciitis. In such cases, molecular analysis is a useful adjunct to avoid unnecessary overtreatment.


2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Senai Goitom Sereke ◽  
Anthony Oriekot ◽  
Felix Bongomin

Abstract Background Thyroid hemiagenesis is a rare congenital anomaly in which one lobe of the thyroid gland fails to develop. There is an increased incidence of associated thyroid disorders in patients with thyroid hemiagenesis. Case presentation A 32-year-old Ugandan woman presented with a complaint of painless neck swelling of 3-months duration. The swelling was associated with a globus sensation. There was no history of thyroid – related problems or treatment prior to this presentation. Physical examination demonstrated a mobile right thyroid swelling without an obvious nodular contour. Neck ultrasound showed an absent left lobe of thyroid gland, a right lobe with a solitary nodule scoring two points on the Thyroid Imaging, Reporting and Data System (TI-RADS) and an isthmus in situ. Extensive search for possible ectopic thyroid tissue was negative. She was biochemically euthyroid. The patient was counseled about thyroid hemiagenesis and was put on a regular follow up in the clinic for the TI-RADS 2 nodule. Conclusion Thyroid hemiagenesis is often associated with other thyroid disorders. Its diagnosis should prompt an active search for other associated morphological or functional thyroid abnormalities.


2020 ◽  
Author(s):  
Xin Guan ◽  
Di Zhang ◽  
Yang Han ◽  
Qingchang Li ◽  
Enhua Wang ◽  
...  

Abstract Background: Lymphoepithelioma-like carcinoma (LELC) is rare in the thymus, which is easy to be misdiagnosed. To improve its clinicopathological knowledge, we describe two cases of thymic LELC, and investigate their clinicopathological features (microscopical and immunohistochemical features), treatment and follow-up data with a review of previously published cases. Case presentation: Two patients in the First Affiliated Hospital of China Medical University underwent complete surgical resection for thymic LELC. In both, they were treated with chemotherapy or radiotherapy after operation. Histologically, tumor cells were arranged in nest-like patterns or stripe-shaped infiltration in collagen fibrous interstitial tissue containing lymphocytes. And the tumor cells were diffusely positive for broad spectrum pan-cytokeratin (CK), CK19, CD5, CD117, epithelial membrane antigen (EMA) and p63, focally positive for CD20, and negative for TdT. Recent clinical reexamine showed that the two patients were alive with no signs of recurrence.Conclusion: We report two cases of thymic LELC with a review of previously published cases to summarize the knowledge of their clinicopathological characteristics, which is necessary for the accurate diagnosis and clinical treatment.


2021 ◽  
Author(s):  
Senai Goitom Sereke ◽  
Anthony Oriekot ◽  
Felix Bongomin

Abstract Background: Thyroid hemiagenesis is a rare congenital anomaly in which one lobe of thyroid gland fails to develop. There is a higher incidence of associated thyroid disorders in patients with thyroid hemiagenesis. Case presentation: A 32-year-old female presented to a hospital with complaint of painless neck swelling of 3-month duration with associated globus sensations. There were no history of thyroid related problems or thyroid related treatment prior to her presentation. Physical examination demonstrated a mobile right thyroid swelling but no obvious nodular contour. Neck ultrasound demonstrated absent left lobe of thyroid gland, right thyroid gland with solitary nodule of TI-RADS 2 and isthmus in situ. Extensive search for possible ectopic thyroid tissue was negative. Thyroid function test was euthyroid. Conclusion: Early detection of this entity and follow up monitoring of thyroid morphologic and hormonal function have paramount importance.


2019 ◽  
Vol 25 (1) ◽  
Author(s):  
Danielle Whiting ◽  
Ian Rudd ◽  
Amit Goel ◽  
Seshadri Sriprasad ◽  
Sanjeev Madaan

Abstract Background Angiomyolipomas are rare mesenchymal tumours arising from the perivascular epithelioid cells consisting of variable amounts of adipose, thick-walled blood vessels and smooth muscle cells. These benign tumours commonly occur in the kidney with only a few case reports of adrenal angiomyolipomas which have the potential to reach a large size and haemorrhage. Case presentation A 45-year-old lady presented with a 3-week history of right loin pain, nausea and vomiting. A CT scan revealed a right adrenal angiomyolipoma measuring 6.3 × 6.8 cm with associated haemorrhage. The lesion was successfully treated with right open adrenalectomy, and histology confirmed the diagnosis of adrenal angiomyolipoma. The patient remained well with no evidence of recurrence at the 36-month follow-up. Conclusion Adrenal angiomyolipomas are rare benign tumours that have the ability to reach a large size and potential to bleed. Here, we report the second case of spontaneous haemorrhage in an adrenal angiomyolipoma, which was successfully treated with open adrenalectomy.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Sara L Weidmayer ◽  
Hakan Demirci

Abstract Background The natural course of a vortex vein varix, though not well understood, has been known to remain stable. However, here we report a novel case of a vortex vein varix that resolved after an extended period of monitoring. Case presentation An asymptomatic 96-year-old Caucasian man was found to have a vortex vein varix. At his previous examination 13 months prior, his fundus was normal. At 13 months of observation, his vortex vein varix become clinically undetectable. Further follow-up confirmed continued absence of the varix. Conclusion This case demonstrates the development then clinical resolution of a vortex vein varix with no clear identifiable factors for its evolution. This case is novel and offers new insight into the natural history of some vortex vein varices, implicating venous congestion as an instigator and venous collateralization as its alleviator, suggesting that vortex vein varices are likely more common than previously reported since some may be temporary and under-identified.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Dalit Porat Ben Amy ◽  
Victoria Yaffe ◽  
Rawan Kawar ◽  
Sharon Akrish ◽  
Imad Abu El-Naaj

Abstract Background Myopericytoma is a rare mesenchymal neoplasm with perivascular myoid differentiation that arises most commonly in middle adulthood. The lesion generally involves the subcutaneous tissue of distal extremities. Myopericytoma of the oral cavity is extremely rare. Herein we report a case of oral myopericytoma in a pediatric patient, who was treated via a conservative approach with a follow up of 8 years. The case is followed by a literature review. To our knowledge this is the first documented case of oral myopericytoma affecting a patient of such a young age. Case presentation A 6 years old boy was referred to the maxillofacial surgery department for the evaluation of a solitary growth of the right maxillary buccal and palatal gingiva. Histology and immunohistochemistry confirmed the diagnosis of myopericytoma. Conclusions Our patient was treated by local excision with no recurrence in 8 years of follow up. Conservative approach should be considered for the treatment oral myopericytoma especially in young patients in tooth bearing areas.


Author(s):  
Zahra Tavoli ◽  
Ali Montazeri

Introduction: Uterus didelphys with obstructed hemivagina associated with ipsilateral renal agenesis (OHVIRA syndrome) is a rare female urogenital malformation and delay in its diagnosis could lead to several complications. Case presentation: A 21-year-old virgin woman was admitted to the emergency department (ED) with severe abdominal pain, without fever and vaginal discharge. She reported a history of cyclic abdominopelvic pain and dysmenorrhea for 5 years. The primary diagnosis (OHVIRA syndrome) was made using ultrasonography, spiral computed tomography (CT) and magnetic resonance imaging (MRI). In addition, laparoscopy was performed to confirm diagnosis and drain hematosalpinx. Then, hysteroscopy was carried out for septum resection and catheter insertion. At one-month follow-up the ultrasonography showed normal left hemicavity of uterus associated with significant decrease in dysmenorrhea. Conclusion: Being aware of OHVIRA syndrome and clinical suspicion of this rare anomaly are essential for making a timely diagnosis, preventing complications, relieving symptoms, and preserving future fertility.


FACE ◽  
2021 ◽  
pp. 273250162110536
Author(s):  
Joshua Harrison ◽  
Samantha Marley ◽  
Shawhin Shahriari ◽  
Christian Bowers ◽  
Anil Shetty

We report a rare case of an extramedullary plasmacytoma (EMP) in the frontal sinus with an indolent clinical presentation. Although a history of trauma was absent, the initial diagnosis was a mucocele, based on the radiological findings. Upon surgical excision, the patient was found to have an EMP. EMP, a form of solitary plasmacytoma, has a significantly high rate of conversion to multiple myeloma. This mandates long-term follow-up, even after successful radiotherapy and/or resection. While radiation therapy is generally considered a first line treatment for EMP, surgical intervention may provide optimal treatment in complicated cases. This case presentation highlights the prognosis of patients diagnosed with EMP.


2021 ◽  
Author(s):  
Tao Sun ◽  
Wentao Wang ◽  
Longshuang He ◽  
Yu Su ◽  
Ning Li ◽  
...  

Abstract Background: Primary trigeminal neuralgia (TN), hemifacial spasm (HFS) and glossopharyngeal neuralgia (GN) are common diseases of nervous system, with similar pathogenesis and treatment strategies. Coexistent of such disease, especially coexistent of TN-HFS-GN simultaneously, is very rare. To date, only nine cases have been reported.Case Presentation: A 70-year-old male with a history of hypertension and diabetes complained of severe involuntary contraction for about 10 years, knife-like and lighting-like pain, which was restricted to the distribution of the second and third branches of trigeminal nerve and pharynx and root of tongue, for about 2 years. Coexistent of TN HFS and GN was diagnosed and MVD was carried out. After MVD, the patient completely free from symptoms and no recurrence and hypoesthesia were recorded in 18 months follow up.Conclusion: Here we report the tenth and oldest male patient with coexistent of TN-HFS-GN. Despite limited reports, MVD is the preferred choice for such diseases which can free patients from spasm and neuralgia.


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