Clinical Characteristics of Nodular Fasciitis of Ear in Children

Abstract Purpose Summarized the clinical characteristics and diagnosis and treatment process of three cases of nodular fasciitis of ear, to provide a basis for clinical diagnosis and treatment. Methods Reviewed the clinical manifestations, images, pathology, treatment and postoperative follow up results of three cases of pediatric nodular fasciitis in the Department of Otorhinolaryngology, Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University from 2018 to 2020. Results The average age at diagnosis were 24 months, with two girls and a boy. Two lesions were found in the left ear and one in the right ear. All cases had a history of biopsy before surgery. Two of three cases showed a sign of rapid growth after biopsy and three of which were ineffective in anti-inflammatory treatment. FISH test for USP6 were performed in two of the three cases with positive results. Three lesions show a hypointensity or isointensity on T1-weighted MRI and a heterogeneous hyperintensity on T2-weighted MRI. ‘‘Fascial tail’’ sign was found on image of all three cases. All lesions underwent surgical resection. Follow-up showed no recurrence and had an intact ear appearance. Conclusion The early misdiagnosis rate of nodular fasciitis of the ear is high. Combine clinical features with imaging findings may improve the accuracy of preoperative diagnosis. Besides the appearance of pathology, USP6 gene test is also an important tool in the diagnosis. The final diagnosis should be based on comprehensive assessment. Complete surgical resection can prevent recurrence.

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Case report: a case of primary pulmonary glomus tumor

10.21203/rs.2.22606/v1 ◽

2020 ◽

Author(s):

shuangshuang Deng ◽

Jianhao Huang ◽

Qi Yin ◽

Jinli Gao ◽

Feilong Wang ◽

...

Keyword(s):

Tumor Recurrence ◽

Glomus Tumor ◽

Clinical Symptoms ◽

Clinical Manifestations ◽

Final Diagnosis ◽

Case Presentation ◽

Glomus Tumors ◽

Disease Case ◽

The Right

Abstract Background Glomus tumors, as extremely rare tumors of the lung, since their rarity and the variety of the clinical symptoms, tend to be misdiagnosed. The location of these tumors as well as their early diagnosis is a pivot for the prognosis of the disease. Case presentation We report a case of old patient whose final diagnosis was primary pulmonary glomus tumor. She initially was found a nodule in the right lung, which was enlarged during follow-up and finally confirmed after surgery. Postoperative pathology was considered as a primary pulmonary glomus tumor. There has been no evidence of tumor recurrence or metastasis so far. Conclusion Primary pulmonary glomus tumor is rare. The clinical manifestations are related symptoms occupying lesions in the lung. The diagnosis of the tumor depends on histopathology and immunohistochemistry. Surgical resection is considered as the most eﬀective treatment for this condition.

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Total thymectomy for thymic lymphoepithelioma-like carcinoma—report of two cases

Surgical Case Reports ◽

10.1186/s40792-019-0706-6 ◽

2019 ◽

Vol 5 (1) ◽

Author(s):

Sachi Kawagishi ◽

Naoko Ose ◽

Masato Minami ◽

Soichiro Funaki ◽

Takashi Kanou ◽

...

Keyword(s):

Surgical Resection ◽

Thymic Carcinoma ◽

Clinical Manifestations ◽

Staging System ◽

Early Recurrence ◽

Anterior Mediastinum ◽

Total Resection ◽

Distant Lymph Node Metastasis ◽

Long Term Follow Up

Abstract Background Thymic carcinoma has been classified into 12 subtypes, thymic lymphoepithelioma-like carcinoma (LELC) is a type of them, and has a pathological organization similar to that of lymphoepithelioma, an undifferentiated type of nasopharyngeal carcinoma. According to a report from the International Thymic Malignancy Interest Group (ITMIG), thymic LELC is a rare tumor and accounts for 6% of all thymic carcinoma cases. We report two cases of surgical resection for thymic LELC and perform a search of other reports of thymic LELC, and clinical manifestations and follow-up data thus obtained are summarized. Case presentation Two patients underwent surgical resection for thymic LELC. In both, tumors were detected in the anterior mediastinum and a total thymectomy was performed. Each was diagnosed with thymic LELC and classified in accordance with the Masaoka staging system as modified stage II. In recent examinations, one patient was doing well after undergoing total resection, whereas early recurrence of distant lymph node metastasis was noted in the other at 5 months after the total resection procedure and died thereafter from a different disease. Conclusion We report two cases of surgical resection for thymic LELC. A successful total resection may positively affect prognosis: thus, long-term follow-up examinations must be performed.

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“Craniotomy Within Craniotomy” Technique for Safe Resection of an Intracranial Arteriovenous Malformation With Frontal Bony Erosion: 2-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opab312 ◽

2021 ◽

Author(s):

MirHojjat Khorasanizadeh ◽

Kristine Ravina ◽

Aristotelis Filippidis ◽

Christopher S Ogilvy

Keyword(s):

Surgical Resection ◽

Endovascular Embolization ◽

Acute Onset ◽

Bone Flap ◽

Intracranial Arteriovenous Malformation ◽

Excessive Bleeding ◽

Left Hand ◽

The Right ◽

Venous Varix

Abstract Surgical resection is one option in the treatment of large high-grade brain arteriovenous malformations (AVMs). Resection of AVMs with skull-eroding components can be challenging due to the risk of excessive bleeding from these components during craniotomy and bone flap removal. We present a case of a 25-yr-old woman who presented with an acute onset right-sided frontal headache. She was found to have a large, frontal Spetzler-Martin grade IV AVM with an associated dural AVM. The AVM had caused focal erosions of the right frontal bone by a venous varix traversing the region of the calvarial defect. An elective staged endovascular embolization followed by surgical resection was recommended considering the patient's young age and the large size of the AVM located in a noneloquent area. Given the high risk of intraoperative hemorrhage during the craniotomy portion of the procedure, a “craniotomy within craniotomy” approach was planned. During this approach, a small rectangle of bone, including the portion eroded by the venous varix, was left in place, while the larger bone flap surrounding it was removed for an initial approach to the AVM. The small bony piece was safely removed at later stages of resection once the arterial feeders had been reasonably obliterated. Immediate postoperative catheter angiogram demonstrated good filling of the intracranial vascular territories with no residual AVM. The patient developed mild left facial and left hand weakness postoperatively, which resolved after 2 wk of follow-up. The patient remained neurologically intact on further follow-up.

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Acute Megakaryocytic Leukemia with or Without Acquired Trisomy 21 in 15 Pediatric Patients

10.21203/rs.3.rs-107199/v1 ◽

2020 ◽

Author(s):

Wenzhi ZHANG ◽

Hui LI ◽

Jingzhen LIU ◽

Jiawei XU ◽

Jinjin HAO ◽

...

Keyword(s):

Trisomy 21 ◽

Poor Prognosis ◽

Clinical Characteristics ◽

Pediatric Patients ◽

Laboratory Data ◽

Clinical Manifestations ◽

The Other ◽

Hematopoietic Stem ◽

Acute Megakaryocytic Leukemia

Abstract The knowledge of clinical characteristics and prognosis of pediatric acute megakaryocytic leukemia (AMKL) with or without acquired +21 was limited. We reported 15 AMKL pediatric patients without Down Syndrome (four cases with acquired +21 and 11 cases without acquired +21) with the clinical manifestations, laboratory data, and prognosis. The clinical features and laboratory data between patients with acquired +21 and patients without acquired +21 are similar. As for prognosis, three of the 11 cases without acquired +21 obtained complete remission (CR) after 1st induction. The median follow-up time of the 11 cases was 9 months. Among four cases with acquired +21, one case gave up treatment during 1st induction, one obtained CR after 1st induction and was still alive after 49 months of follow-up. One case obtained CR after 2nd induction and was still alive for 15 months of follow-up after bone marrow transplantation, the other patient was planning for allogeneic hematopoietic stem cell transplantation (HSCT) without CR. The median follow-up time of the four cases was 12 months. None relapsed in our study. In conclusion, acquired trisomy 21 may not be an indicator for poor prognosis. Cytogenetics analysis can help us for diagnosis stratification, prognostic judgment and individualized treatment of AMKL.

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Recurrent giant cell tumour of the maxillary sinus and pterygoid process treated with denosumab

BMJ Case Reports ◽

10.1136/bcr-2018-225095 ◽

2018 ◽

pp. bcr-2018-225095

Author(s):

Duarte Rosa ◽

Raquel Baptista Dias ◽

João Cunha Salvador ◽

Alexandra Borges

Keyword(s):

Maxillary Sinus ◽

Giant Cell ◽

Giant Cell Tumour ◽

Neck Surgery ◽

Cell Tumour ◽

Pterygoid Process ◽

Bone Expansion ◽

The Right ◽

Denosumab Treatment

We report the case of a 74-year-old man with a giant cell tumour (GCT) of the right maxilla and pterygoid process. The patient presented to the maxillofacial and head and neck surgery clinic with an ulcerated lesion of the hard palate. Initial workup with CT revealed a mass within the right maxillary sinus and pterygoid process with associated bone expansion and erosion. Biopsy showed a GCT with mucosal ulceration. Two years after surgical resection, a follow-up CT revealed tumour recurrence involving the right pterygoid process and lateral pterygoid muscle. The patient was then proposed for therapy with denosumab. Under denosumab treatment, the lesion maintained stable dimensions and became sclerotic and heavily ossified.

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Sight-threatening progressive corneo-scleral involvement in porphyria cutanea tarda

BMJ Case Reports ◽

10.1136/bcr-2021-245160 ◽

2021 ◽

Vol 14 (10) ◽

pp. e245160

Author(s):

Sonali Prasad ◽

Vidhata Vidhata ◽

Subhash Prasad

Keyword(s):

Porphyria Cutanea Tarda ◽

Clinical Manifestations ◽

Ocular Involvement ◽

Slit Lamp ◽

Uroporphyrinogen Decarboxylase ◽

Progressive Loss ◽

The Right ◽

Lost To Follow Up ◽

Test Serum

Porphyria cutanea tarda is the most common type of porphyria. It is associated with a deficiency of uroporphyrinogen decarboxylase enzyme responsible for heme synthesis. Clinical manifestations are predominantly dermatological and very rarely present with ocular involvement. Although scleral thinning in the interpalpebral area is a well-documented entity, sight-threatening corneal involvement is rarely described. We, herein report a case of a 58-year-old man who presented with ocular surface dryness, photophobia and mild redness. Slit-lamp biomicroscopy revealed corneo-scleral thinning in both eyes. The diagnosis was confirmed with a urine porphyrin test, serum iron and serum ferritin levels. We started him on conservative management after which he was lost to follow-up. He presented again after 6 years with total corneal opacification and progressive loss of vision in the right eye.

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Case of primary hepatic leiomyosarcoma successfully treated with laparoscopic right hepatectomy

BMJ Case Reports ◽

10.1136/bcr-2019-233567 ◽

2020 ◽

Vol 13 (2) ◽

pp. e233567 ◽

Cited By ~ 1

Author(s):

Stephanie Vella ◽

Kelvin Cortis ◽

David Pisani ◽

James Pocock ◽

Luca Aldrighetti

Keyword(s):

Physical Examination ◽

Surgical Resection ◽

Epigastric Pain ◽

Large Mass ◽

Disease Recurrence ◽

Right Hepatectomy ◽

Liver Lobe ◽

Laparoscopic Right Hepatectomy ◽

The Right

We describe the case of a 77-year-old woman, presenting with non-specific epigastric pain. Physical examination and subsequent imaging revealed the presence of a large mass in the right liver lobe. This was shown to be a leiomyosarcoma on biopsy histology. Further investigation confirmed this to be a primary hepatic leiomyosarcoma with no evidence of metastases. The patient underwent successful surgical resection. She is currently under imaging follow-up, with no evidence of disease recurrence.

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31 Intracranial growing teratoma syndrome (IGTS): An international retrospective study

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2018.295 ◽

2018 ◽

Vol 45 (S3) ◽

pp. S13-S13

Author(s):

George Michaiel ◽

Douglas Strother ◽

Nicholas Gottardo ◽

Ute Bartels ◽

Hallie Coltin ◽

...

Keyword(s):

Surgical Resection ◽

Clinical Characteristics ◽

Progressive Disease ◽

Mature Teratoma ◽

Male Gender ◽

Cell Tumor ◽

Total Resection ◽

Beta Hcg ◽

Median Serum

BACKGROUND: IGTS is a rare phenomenon of paradoxical germ cell tumor (GCT) growth during or following treatment despite normalization of tumor markers. We sought to evaluate the frequency, clinical characteristics and outcome of IGTS in patients in 21 North-American and Australian institutions. METHODS: Patients with IGTS diagnosed from 2000-2017 were retrospectively evaluated. RESULTS: Out of 739 GCT diagnoses, IGTS was identified in 33 patients (4.5%). IGTS occurred in 9/191 (4.7%) mixed-malignant GCTs, 4/22 (18.2%) immature teratomas (ITs), 3/472 (0.6%) germinomas/germinomas with mature teratoma, and in 17 secreting non-biopsied tumours. Median age at GCT diagnosis was 10.9 years (range 1.8-19.4). Male gender (84%) and pineal location (88%) predominated. Of 27 patients with elevated markers, median serum AFP and Beta-HCG were 70 ng/mL (range 9.2-932) and 44 IU/L (range 4.2-493), respectively. IGTS occurred at a median time of 2 months (range 0.5-32) from diagnosis, during chemotherapy in 85%, radiation in 3%, and after treatment completion in 12%. Surgical resection was attempted in all, leading to gross total resection in 76%. Most patients (79%) resumed GCT chemotherapy/radiation after surgery. At a median follow-up of 5.3 years (range 0.3-12), all but 2 patients are alive (1 succumbed to progressive disease, 1 to malignant transformation of GCT). CONCLUSION: IGTS occurred in less than 5% of patients with GCT and most commonly after initiation of chemotherapy. IGTS was more common in patients with IT-only on biopsy than with mixed-malignant GCT. Surgical resection is a principal treatment modality. Survival outcomes for patients who developed IGTS are favourable.

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Epibulbar Nodular Fasciitis

Case Reports in Ophthalmology ◽

10.1159/000445974 ◽

2016 ◽

Vol 7 (1) ◽

pp. 262-267 ◽

Cited By ~ 3

Author(s):

David John Massop ◽

Paul Adam Frederick ◽

He J. Li ◽

Amy Lin

Keyword(s):

Surgical Resection ◽

Current Literature ◽

Excisional Biopsy ◽

Nodular Fasciitis

Purpose: To report a case of epibulbar nodular fasciitis in a 32-year-old female and provide context by reviewing the current literature. Results: Using excisional biopsy, the patient was successfully diagnosed and treated for epibulbar nodular fasciitis. Upon follow-up, there has been no recurrence, consistent with the typical course for nodular fasciitis. Conclusions: Epibulbar nodular fasciitis is a rare process that can be successfully treated by surgical resection. While two cases of trauma-associated epibulbar nodular fasciitis have been present in the literature, our case did not have such a history. The etiology of nodular fasciitis remains unclear.

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A Retrospective Study of Superficial Type Atypical Lipomatous Tumor

Frontiers in Medicine ◽

10.3389/fmed.2020.609515 ◽

2020 ◽

Vol 7 ◽

Author(s):

Emi Mashima ◽

Yu Sawada ◽

Natsuko Saito-Sasaki ◽

Kayo Yamamoto ◽

Shun Ohmori ◽

...

Keyword(s):

Surgical Resection ◽

Tumor Size ◽

Clinical Characteristics ◽

Clinical Manifestations ◽

Tumor Location ◽

Atypical Lipomatous Tumor ◽

Lipomatous Tumor ◽

Physical Examinations ◽

Superficial Type ◽

Well Differentiated

Atypical lipomatous tumor (ALT) has been defined as a well-differentiated liposarcoma exhibiting a higher frequency of a local recurrence after surgical resection. ALT is mainly classified into deep type and superficial type. Compared with deep type ALT, superficial type ALT is rarely observed. One of the most important issues is that little has been known about superficial type ALT and it is not easy to predict the presence of superficial type ALT before surgical resection. To clarify the clinical manifestations of superficial type ALT, we examined 15 cases with superficial type ALT and 118 cases with benign lipoma, and analyzed their differences in clinical characteristics and the findings of MRI test. In clinical characteristics, the tumor size of superficial type ALT was significantly greater than that of benign lipoma, and superficial type ALT showed a significantly higher frequency of the tumor size of more than 4 cm. Superficial type ALT exhibited poor tumor mobility and hardness with elastic soft. In addition, a significantly higher frequency of tumor location of superficial type ALT was observed in extremities. Among tumor sites at the trunk, buttocks, and shoulder were high frequent location in superficial type ALT. In an MRI examination, superficial type ALT exhibited a significantly higher frequency of the septal structures compared with benign lipoma. The combinations of clinical characteristics, including physical examinations, MRI, and histological examinations, are helpful for the diagnosis of superficial type ALT.

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