scholarly journals Plexiform Neurofibroma Involving the Lacrimal Gland

2017 ◽  
Vol 8 (1) ◽  
pp. 67-72 ◽  
Author(s):  
Mikael Hofsli ◽  
Nico Gampenrieder ◽  
Steffen Heegaard
Keyword(s):  
Lacrimal Gland ◽  
Histopathological Examination ◽  
Plexiform Neurofibroma ◽  
Neurofibromatosis Type ◽  
High Recurrence Rate ◽  
Magnetic Resonance Imaging Scan ◽  
Upper Eyelid ◽  
Nerve Branch ◽  
First Case ◽  
The Right

Background: To present a rare case of a 2-year-old girl with neurofibromatosis type 1 (NF1) who presented with ptosis of the right upper eyelid along with a tumor in the eyelid. Methods: A magnetic resonance imaging scan of the orbit revealed a solid tumor located extraconally at the site of the right lacrimal gland. A transcranial orbitotomy was performed. Results: Histopathological examination demonstrated expanded nerve branches/fascicles cut in various planes in between normal lacrimal gland acini. These findings were consistent with a plexiform neurofibroma presumably deriving from the lacrimal nerve and/or a supraorbital nerve branch. Conclusion: This is the first case of a plexiform neurofibroma involving the lacrimal gland ever described and the tumor shows similarities with neurofibroma in other salivary glands with a high recurrence rate. Plexiform neurofibromas are frequently seen in patients with NF1 and rarely undergo malignant transformation.

scholarly journals An Exceptional Case of Intraparotid Plexiform Neurofibroma Originating from Autonomic Fibers of the Auriculotemporal Nerve

2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Sarantis Blioskas ◽  
Sotiris Sotiriou ◽  
Katerina Rizou ◽  
Triantafyllia Koletsa ◽  
Petros Karkos ◽  
...  
Keyword(s):  
Parotid Gland ◽  
Plexiform Neurofibroma ◽  
Surgical Excision ◽  
Neurofibromatosis Type ◽  
Exceptional Case ◽  
Benign Tumors ◽  
First Case ◽  
Superficial Lobe ◽  
The Right ◽  
Auriculotemporal Nerve

Plexiform neurofibromas are benign tumors that tend to occur in patients suffering from neurofibromatosis type 1 (NF-1). This report addresses a rare case where the tumor affected the parotid gland, deriving almost exclusively from the peripheral portion of the facial nerve. A 6-year-old male was referred to us complaining about a gradually enlarging swelling over the right parotid area. Imaging localized the lesion to the superficial lobe of the parotid gland, suggesting a neurofibroma. Cosmetic disfigurement and a functional deficit led us to perform complete surgical resection. Meticulous surgical dissection as well as auriculotemporal nerve origin made complete extirpation possible with almost zero morbidity and ensured alleviation of both aesthetic impairment and pain. This is the first case of an intraparotid PN in a pediatric NF-1 patient, which originated from branches of the auriculotemporal nerve and particularly from fibers of the autonomic nervous system. Radical surgical excision was decided according to established decision-making algorithms.

scholarly journals Primary Neuroendocrine Carcinoma of Ocular Adnexa

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Daisuke Yamanouchi ◽  
Toshiyuki Oshitari ◽  
Yosuke Nakamura ◽  
Jiro Yotsukura ◽  
Kaoru Asanagi ◽  
...  
Keyword(s):  
Neuroendocrine Carcinoma ◽  
Lacrimal Gland ◽  
Histopathological Examination ◽  
Combined Therapy ◽  
Surgical Excision ◽  
Excision Biopsy ◽  
Upper Eyelid ◽  
Ocular Adnexa ◽  
Complete Surgical Excision ◽  
The Right

We present our findings in a case of primary neuroendocrine carcinoma (NEC) of the lacrimal gland and a case of primary Merkel cell carcinoma (MCC) of the eyelid. An 86-year-old man noticed a swelling of the left upper eyelid three months earlier. We performed excision biopsy and histopathological examination indicated that he had a primary NEC of the left lacrimal gland. He underwent chemotherapy followed by excision including the clinically visible margins and 50 Gy radiotherapy of the surgical margins. He had neither recurrence nor metastasis for 6 months since the last radiotherapy. An 80-year-old man noticed a nodule in the right upper eyelid and was referred to our hospital because the size was increasing rapidly. A complete surgical excision of the margins of the tumor was performed with histopathological confirmation of negative margins. The final diagnosis was a primary MCC of the right upper eyelid. After surgery, he underwent 50 Gy radiotherapy on the neck to prevent metastasis. No recurrence or metastasis was found for two years. Although primary NEC of the ocular adnexa is extremely rare, the tumor has high malignancy and readily metastasizes. Thus, combined therapy including surgery, radiotherapy, and/or chemotherapy is needed for complete management of NEC.

scholarly journals Langerhans cell histiocytosis of the orbit and frontal sinus of the adult woman: A first case report in Poland

2019 ◽  
Vol 10 ◽  
pp. 234
Author(s):  
Kacper Kostyra ◽  
Bogusław Kostkiewicz
Keyword(s):  
Case Report ◽  
Frontal Sinus ◽  
Langerhans Cell Histiocytosis ◽  
Histopathological Examination ◽  
Langerhans Cell ◽  
Radical Excision ◽  
Multisystem Disease ◽  
Upper Eyelid ◽  
First Case ◽  
The Right

Background: Langerhans cell histiocytosis (LCH) is a term describing a clonal proliferation of pathologic Langerhans cells (histiocytes), which may manifest as unisystem (unifocal or multifocal) or multisystem disease. LCH is a rare cause of the orbital tumor with the predilection to its lateral wall which is particularly common in children. Case Description: We report an unusual case of a 33-year-old woman, 6 months after childbirth, who presented with the edema of the right orbit and upper eyelid with headaches. On physical examination, the patient had a right superior and lateral swelling of the eyelid and the orbit and right enophthalmos, without blurred vision. Magnetic resonance imaging showed well-defined, expansile, intensely homogeneously enhancing mass lesion in the right superolateral orbital rim with the destruction of the upper wall of the orbit, growing into the frontal sinus and frontal part of the cranium with the bold of the dura mater in this region. Radical excision of the tumor was achieved through a right fronto-temporo-orbito-zygomatic craniotomy. Histopathological examination had confirmed the diagnosis of the LCH. The patient was discharged home with a modified Rankin Scale score of 0. Conclusion: The main purpose of this case report is that LCH should be considered as one of the possible causes of quickly appearing tumor of the orbit in adults.

scholarly journals Alternative Leprosy Treatment Using Rifampicin Ofloxacin Minocycline (ROM) Regimen – Two Case Reports

2019 ◽  
Vol 11 (3) ◽  
pp. 89-93
Author(s):  
Yohanes Widjaja ◽  
Khairuddin Djawad ◽  
Saffruddin Amin ◽  
Widyawati Djamaluddin ◽  
Dirmawati Kadir ◽  
...  
Keyword(s):  
Clinical Improvement ◽  
Alternative Treatment ◽  
Histopathological Examination ◽  
Case Reports ◽  
Erythematous Plaque ◽  
First Case ◽  
Satisfactory Outcome ◽  
Significant Clinical Improvement ◽  
The Right

Abstract Introduction. Leprosy is a disease that predominantly affects the skin and peripheral nerves, resulting in neuropathy and associated long-term consequences, including deformities and disabilities. According to the WHO classification, there are two categories of leprosy, paucibacillary (PB) and multibacillary (MB). The standard treatment for leprosy employs the use of WHO MDT (Multi Drug Treatment) regimen, despite its multiple downsides such as clofazimine-induced pigmentation, dapsone-induced haematological adverse effects, poor compliance due to long therapy duration, drug resistance, and relapse. Multiple studies and case reports using ROM regimen have reported satisfactory results. Nevertheless, there are still insufficient data to elucidate the optimum dosage and duration of ROM regimen as an alternative treatment for leprosy. Previous experience from our institution revealed that ROM regimen given three times weekly resulted in a satisfactory outcome. Case Reports. We report two cases of leprosy treated with ROM regimen from our institution. The first case was PB leprosy in a 64-year-old male who presented with a single scaly plaque with erythematous edge on the right popliteal fossa. Sensibility examination showed hypoesthesia with no peripheral nerve enlargement. Histopathological examination confirmed Borderline Tuberculoid leprosy. ROM regimen was started three times weekly for 6 weeks and the patient showed significant clinical improvement at the end of the treatment with no reaction or relapse until after 6 months after treatment. The second case was MB leprosy in a 24-year-old male patient with clawed hand on the 3rd-5th phalanges of the right hand and a hypoesthetic erythematous plaque on the forehead. Histopathology examination confirmed Borderline leprosy. The patients received ROM therapy 3 times a week with significant clinical improvement after 12 weeks. Conclusion. ROM regimen given three times weekly for 6 weeks in PB leprosy and 12 weeks in MB leprosy resulted in a significant clinical improvement. Thus, ROM regimen could be a more effective, safer, faster alternative treatment for leprosy.

scholarly journals Surgery Combined With Embolization in the Treatment of Plexiform Neurofibroma: A Case Report and Literature Review

2021 ◽  
Vol 6 (4) ◽  
pp. 01-04
Author(s):  
Reinaldo Filho
Keyword(s):  
Plexiform Neurofibroma ◽  
Arterial Embolization ◽  
Neurofibromatosis Type ◽  
Self Esteem ◽  
Life Threatening ◽  
Feeding Vessels ◽  
The Right ◽  
Polyvinyl Alcohol Particles

Neurofibromatosis type 1 (NF1) is an autosomal dominant multisystem genodermatosis resulting from a mutation on chromosome 17q11.2, characterized by diverse clinical expression with involvement of the skin, nervous system, bones, eyes and other systems. Neurofibromas are tumors located along nerve bundles; when they become large, they are called plexiform neurofibromas. Complications of neurofibromas are rare, but include malignant transformation and potentially life-threatening hemorrhages. A comprehensive perioperative management, including arterial embolization of feeding vessels, is required in order to perform a safe surgical procedure and to reduce potential surgical complications. We report a case of a large plexiform neurofibroma on the right flank, treated in a two-stage approach: first, embolization with polyvinyl alcohol particles of the inferior epigastric, lumbar and superior gluteal arteries on the right and, second, 24 hours after embolization, exeresis of the lesion, which weighed 2.5 kg. Two years after surgery, the patient was asymptomatic, without tumor recurrence and with an important improvement in self-esteem and quality of life.

scholarly journals Hyperfunctioning Solid/Trabecular Follicular Carcinoma of the Thyroid Gland

2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Luca Giovanella ◽  
Fabrizio Fasolini ◽  
Sergio Suriano ◽  
Luca Mazzucchelli
Keyword(s):  
Thyroid Carcinoma ◽  
Follicular Thyroid Carcinoma ◽  
Histopathological Examination ◽  
Thyroid Tissue ◽  
Follicular Carcinoma ◽  
First Case ◽  
Right Lobe ◽  
Rare Malignancy ◽  
The Right ◽  
Trabecular Carcinoma

A 68-year-old woman with solid/trabecular follicular thyroid carcinoma inside of an autonomously functioning thyroid nodule is described in this paper. The patient was referred to our clinic for swelling of the neck and an increased pulse rate. Ultrasonography showed a slightly hypoechoic nodule in the right lobe of the thyroid. Despite suppressed TSH levels, the -pertechnetate scan showed a hot area corresponding to the nodule with a suppressed uptake in the remaining thyroid tissue. Histopathological examination of the nodule revealed a solid/trabecular follicular thyroid carcinoma. To the best of our knowledge, this is the first case of hyperfunctioning follicular solid/trabecular carcinoma reported in the literature. Even if a hyperfunctioning thyroid carcinoma is an extremely rare malignancy, careful management is recommended so that a malignancy will not be overlooked in the hot thyroid nodules.

Spontaneous Feline Pneumothorax Caused by Ruptured Pulmonary Bullae Associated With Possible Bronchopulmonary Dysplasia

2010 ◽  
Vol 46 (2) ◽  
pp. 138-142 ◽  
Author(s):  
Marjorie E. Milne ◽  
Christina McCowan ◽  
Ben P. Landon
Keyword(s):  
Computed Tomography ◽  
Case Report ◽  
Bronchopulmonary Dysplasia ◽  
Spontaneous Pneumothorax ◽  
Histopathological Examination ◽  
Lung Lobe ◽  
First Case ◽  
Adult Cat ◽  
The Right ◽  
Lung Lobes

Spontaneous pneumothorax is rarely reported in the cat. This case report describes the use of computed tomography (CT) to diagnose pulmonary bullae in an adult cat with recurrent spontaneous pneumothorax. A large bulla in the right middle lung lobe and several blebs in other lobes were identified by CT. Partial lobectomy of the right middle and right and left cranial lung lobes was successfully performed to remove the affected portions of lung. Histopathological examination suggested bronchopulmonary dysplasia (BPD) as the underlying cause for development of the pulmonary bulla. This is the first case report in the veterinary literature describing the use of CT to identify pulmonary bullae in the cat with BPD as a possible underlying cause.

scholarly journals A Case of Giant Posterior Mediastinal Mass: Gastrointestinal Stromal Tumor Developed in Neurofibromatosis Type 1 Patient.

2021 ◽  
Author(s):  
Volkan ASLAN ◽  
Fatih Gürler ◽  
ozan yazıcı ◽  
özlem erdem ◽  
ali çelik ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Genetic Disorders ◽  
Neurofibromatosis Type ◽  
Skin Lesions ◽  
Posterior Mediastinum ◽  
Pathological Examination ◽  
Imatinib Treatment ◽  
First Case ◽  
Posterior Mediastinal

Abstract Introduction: Gastrointestinal Stromal Tumors mostly located in the stomach and esophageal GIST is extremely rare. Various genetic disorders increase the risk of developing GIST, among these, NF-1 is the most common. A case of posterior mediastial GIST developing in the neurofibromatosis patient has not been reported yet.Case: A 48-year-old male patient is being evaluated with complaints of dyspnea and progressive dysphagia. Computed tomography detected a cystic, lobulated contour mass that was located at the posterior mediastinum and measuring 11 x 14x12 cm. The giant semisolid mass has been dissected from adjacent the vertebra, the esophagus, pericardium and inferior pulmonary vein and Histopathological examination revealed GİST. Pathological examination of skin lesions confirmed NF-1. Finally, NF-1-associated posterior mediastinal GIST was diagnosed and adjuvant imatinib treatment was initiated. Conclusion: To the best our knowledge, this patient was the first case, who diagnosed with posterior mediastinal GIST developing on the basis of NF-1.

Intravenous leiomyomatosis: the first reported case of intraoperative intracaval embolisation of tumour to the right atrium

2020 ◽  
Vol 13 (3) ◽  
pp. e233341
Author(s):  
Gillian A Corbett ◽  
Catherine O'Gorman ◽  
Waseem Kamran
Keyword(s):  
Right Atrium ◽  
Uterine Leiomyoma ◽  
Histopathological Examination ◽  
Vena Cava ◽  
Abdominal Distension ◽  
Preoperative Imaging ◽  
Intravenous Leiomyomatosis ◽  
Midline Laparotomy ◽  
First Case ◽  
The Right

Intravenous leiomyomatosis is extremely rare. This case describes a 42-year-old woman who presented with abdominal distension, cyclical bloating and urinary retention. Preoperative imaging showed a multilobulated uterine mass. Following multidisciplinary team discussion, a complete staging surgery consisting of midline laparotomy, total hysterectomy and bilateral salpingo-oophrectomy was performed. Intraoperatively, a large multilobulated uterine mass was noted with engorgement of the infundibulopelvic ligaments due to intravascular extension of tumour. On removal of the uterus, the patient desaturated and became hypotensive. Intraoperative transoesophageal echocardiography revealed mass extending from the inferior vena cava (IVC) into the right atrium (RA). The cardiothoracic surgical team retrieved a worm-like mass extending from the IVC into the RA. Histopathological examination diagnosed a large uterine leiomyoma with intravenous leiomyomatosis. The mass from the RA was a bland spindle cell tumour which matched the uterine mass histopathologically. Intravenous leiomyomatosis is a rare variant of uterine leiomyoma. Although intracardiac extension has been described, this is the first case of intraoperative embolisation of pelvic tumour to the RA at hysterectomy.

Congenital giant plexiform neurofibroma with occipital calvarial dysplasia in association with meningoencephalocele in neurofibromatosis Type 1 and segmental neurofibromatosis

2013 ◽  
Vol 12 (5) ◽  
pp. 458-464 ◽  
Author(s):  
Ravi Dadlani ◽  
Venkatraman Sadanand ◽  
Nandita Ghosal ◽  
Alangar S. Hegde
Keyword(s):  
Plexiform Neurofibroma ◽  
Neurofibromatosis Type ◽  
Transverse Sinus ◽  
Calvarial Defect ◽  
Review Of Literature ◽  
Radiological Findings ◽  
First Case ◽  
Rare Lesion ◽  
Vascular Channels

Giant plexiform neurofibroma (GPNF) of the scalp is an extremely rare lesion reported in association with neurofibromatosis. Occipital location of GPNF is even more infrequent, especially in association with occipital dysplasia (OD). The authors report 2 pediatric cases of GPNF associated with OD. The first case had an associated meningoencephalocele, and the second had large vascular channels within the lesion and the dominant ipsilateral transverse sinus lying in the center of the calvarial defect. The authors present these 2 unusual cases with a review of literature and discuss the radiological findings, theories of etiopathogenesis of the OD, and management dilemmas.

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