scholarly journals Soft tissue Rosai-Dorfman disease of the posterior mediastinum

2009 ◽  
Vol 35 (7) ◽  
pp. 717-720 ◽  
Author(s):  
Antônio Luiz Penna Costa ◽  
Natália Oliveira e Silva ◽  
Marina Pamponet Motta ◽  
Rodrigo Abensur Athanazio ◽  
Daniel Abensur Athanazio ◽  
...  

Rosai-Dorfman disease (RDD) consists of sinus histiocytosis with massive lymphadenopathy. Extranodal involvement occurs in up to 43% of cases. However, isolated soft tissue RDD is rare. Isolated mediastinal RDD is exceedingly rare, and there have been only three previous reports. Involvement of the posterior mediastinum in RDD has been reported only in the context of disseminated RDD. Here, we report the case of a 49-year-old female patient with a two-year history of cervical pain and lymphadenomegaly, which resolved spontaneously. A CT scan revealed a left paravertebral mass with a diameter of 6 cm. The patient was submitted to surgical excision of the mass. Microscopic examination and immunophenotyping of the surgical specimen led to a diagnosis of RDD. During a 12-month follow-up period, the patient complained of mild cough and chest pain. Periodic imaging tests showed no sign of recurrence, and no postoperative cervical lymphadenomegaly was detected.

2018 ◽  
Vol 17 (4) ◽  
pp. 452 ◽  
Author(s):  
Rubyath C. Rajib ◽  
Rajasekharan Pillai ◽  
Ibrahim A. Sulaiman ◽  
Ibrahim Al-Haddabi

Rosai-Dorfman disease (RDD) is a rare benign proliferative histiocytic disorder characterised by massive lymphadenopathy. While extranodal involvement can occur in generalised RDD, isolated soft tissue RDD (STRDD) is extremely rare. We report a 17-year-old male patient who presented to the maxillofacial outpatient department of the Sultan Qaboos Hospital, Salalah, Oman, in 2015 with a painless cheek mass which had been slowly growing over the previous two months. Routine histopathological examinations and immunohistochemistry confirmed a diagnosis of STRDD. Currently, surgical excision is considered to be the most effective curative treatment for STRDD, as the outcomes of other treatment modalities are still unknown. Despite its rarity, STRDD should be considered in the differential diagnosis of histiocytic soft tissue lesions.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Chiara Pagnoni ◽  
Luca Improta ◽  
Rossana Alloni ◽  
Francesco Mallozzi Santa Maria ◽  
Irene Aprile ◽  
...  

Abstract Background Myxofibrosarcoma (MFS) is a rare soft tissue sarcoma with a high recurrence rate and a low risk of distant metastasis. It occurs mainly in the extremities of elderly men. Head and neck MFS is extremely rare. Surgery is the cornerstone of treatment. The role of radiotherapy (RT) and chemotherapy (CHT) on MFS is still debated. Case presentation A 67-year-old Caucasian man presented to our sarcoma referral center (SRC) with a history of MFS of the neck excised with microscopic positive surgical margins in a non-referral center. Staging imaging exams did not reveal distant metastasis. After a multidisciplinary discussion, preoperative RT was administered with a total dose of 50 Gy followed by wide surgical excision. Histological examination was negative for viable tumor cells. No relapse occurred during the 24-month postoperative follow-up. Conclusions The case described suggests the importance of planned combined treatments with both RT and surgery for high-grade soft tissue sarcoma. RT seems to be promising within this specific histotype. Close follow-up is advisable in all cases. Further studies are needed to confirm if the observed efficacy of combined treatments results in a prolonged time of disease-free survival and overall survival.


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.


2014 ◽  
Vol 3 (2) ◽  
pp. 45-48 ◽  
Author(s):  
Shyamal Chandra Debnath ◽  
Suman Kumar Roy ◽  
RR Kairy

Glomus tumor is a rare condition and constitutes only 1 % of all soft tissue tumor of body. Glomus tumor is familiar for its unusual presentation and long standing symptoms due to delay in diagnosis. Authors have presented the clinical behavior and treatment of 7 patients with subungual soft tissue mass. Following surgical excision, all patients cured of symptoms, at the end of follow up, no recurrence occurred; post operative nail deformity was insignificant. DOI: http://dx.doi.org/10.3329/bdjps.v3i2.18250 Bangladesh Journal of Plastic Surgery July 2012, 3(2): 45-48


2020 ◽  
Vol 11 (4) ◽  
pp. 5187-5191
Author(s):  
Sivapriya G Nair ◽  
Jina Raj ◽  
Sajesh K Menon ◽  
Suhas Udayakumaran ◽  
Roshni P R

Rosai Dorfman disease is a rare histiocytic disorder. It is also known as Sinus Histiocytosis. It is with massive lymphadenopathy involves an overproduction of a type of white blood cell. The disease is rarely associated with intracranial and intraorbital involvement. Intracranial Rosai-Dorfman can mimic meningioma. Other pathologies also underline its pathologies. Here, we report a nine-year-old boy with a history of proptosis of the right eye and presenting with multiple skull lesions. Histopathological study revealed Sphenopetroclival lesion, which features that of Rosai Dorfman Disease. His MRI scan of the brain was taken, which showed evidence of right optic nerve meningioma with sella and suprasellar extension, causing severe proptosis. The child underwent right frontotemporal craniotomy with petrosectomy and Transylvanian, subtemporal approach to multicompartmental Rosai-Dorfmans lesion. After four months, the patient had a recurrence of the disease on which chemotherapy and steroids were started, which also did not show much response while taking an MRI scan. A corticosteroid is a useful option in the Central Nervous System Rosai Dorfman disease treatment. But this patient showed a negative outcome to the treatment.


2013 ◽  
Vol 7 (9-10) ◽  
pp. 651 ◽  
Author(s):  
Gabriele Guglielmetti ◽  
Paolo De Angelis ◽  
Paolo Mondino ◽  
Carlo Terrone ◽  
Alessandro Volpe

Perivascular Epithelioid Cell tumour (PEComa) is rare. We describe a 39-year-old man who underwent a left radical orchidectomy and adjuvant radiation therapy for a stage IA classical testicular seminoma. He was diagnosed with a mass lateral to the right common iliac artery that was considered suspicious for late lymph node relapse after 3 years of follow-up. Due to the unusual location of the mass and the equivocal findings of percutaneous biopsy, a laparoscopic pelvic lymphadenectomy was performed. Final pathology revealed PEComa of soft tissue. The patient is disease free after 38 months of follow-up without adjuvant treatment. The presence of rare soft-tissue neoplasm should be considered in differential diagnosis of retroperitoneal masses during follow-up of germ cell tumours. Suspicious isolated recurrences of these neoplasms in unusual locations can require surgical excision to confirm diagnosis and avoid inappropriate treatment.


2014 ◽  
Vol 10 (4) ◽  
pp. 91-94
Author(s):  
A Bhatnagar ◽  
M Deshpande

Servelle Martorelle Syndrome is a congenital vascular malformation associated with soft tissue hypertrophy and bony hypoplasia. This rarely involves whole of an extremity, with involvement of part of limbs reported in literature. We present a case of a twelve year boy who presented to the Department of Plastic Surgery SGPGIMS in April 2011 ,with history of circumferential soft tissue hypertrophy involving whole of left upper limb, scapular region and axilla since birth. The entire left upper limb length was lesser than the right upper limb. Hence this is a very rare case of Servelle Martorelle Syndrome having extensive limb involvement at a very young age. Highlighted is the role of conservative treatment and close follow-up to understand the natural history of the diseases, with prompt treatment of complications. DOI: http://dx.doi.org/10.3126/kumj.v10i4.11011 Kathmandu Univ Med J 2012;10(4):91-94


2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Jong-Ho Nam ◽  
Jong-Seon Park ◽  
Joon Hyuk Choi

Abstract Background Paragangliomas are tumors that arise from extra-adrenal chromaffin cells. Herein, we present a rare case of a functional paraganglioma in the posterior mediastinum. Case presentation A 36-year-old man presented with paroxysms of chest pain and headache. At presentation, the patient’s blood pressure was 190/120 mmHg. Chest computed tomography and magnetic resonance imaging revealed a left paravertebral mass in the posterior mediastinum. 123I-metaiodobenzylguanidine scanning revealed focally high tracer uptake in the left paravertebral area. The metanephrine level in the urine was elevated, confirming a rare, catecholamine-producing, functional paraganglioma in the posterior mediastinum. Before surgery, the patient was prepared by orally administering α- and β-adrenergic blockers. The mass was then resected via a lateral thoracotomy. The metanephrine level in urine was normal 24 h after surgery. Conclusions Paragangliomas in the posterior mediastinum are very rare, but more than half of all cases are functional. The associated symptoms are curable with complete resection, and long-term follow-up for recurrence is important.


2012 ◽  
Vol 2 (1) ◽  
pp. 29 ◽  
Author(s):  
James Moloney ◽  
John Drumm ◽  
Deirdre M. Fanning

Soft-tissue sarcomas of the genitourinary tract account for only 1-2% of urological malignancies and 2.1% of soft-tissue sarcomas in general. A 69-year-old male complained of a 4 month history of a painless right groin swelling during routine urological review for prostate cancer follow-up. Clinical examination revealed a non-tender, firm right inguinoscrotal mass. There was no discernible cough impulse. Computed tomography of abdomen and pelvis showed a non-obstructed right inguinal hernia. During elective hernia repair a solid mass involving the spermatic cord and extending into the proximal scrotum was seen. The mass was widely resected and a right orchidectomy was performed. Pathology revealed a paratesticular sarcoma. He proceeded to receive adjuvant radiotherapy. Only around 110 cases of leiomyosarcoma of the spermatic cord have been described in the literature. They commonly present as painless swellings in the groin. The majority of diagnoses are made on histology.


Rare Tumors ◽  
2010 ◽  
Vol 2 (1) ◽  
pp. 5-7 ◽  
Author(s):  
Munenori Ide ◽  
Takayuki Asao ◽  
Takatomo Yoshida ◽  
Junko Hirato ◽  
Tatsuo Shimura ◽  
...  

Rosai-Dorfman disease (RDD) was formerly known as “sinus histiocytosis with massive lymphadenopathy”, and cases involving the gastrointestinal tract are rare. We present a case of pure extranodal RDD, resected as a polypoid lesion in colonoscopic study. The patient was a 62-year old woman with a history of sigmoidectomy for unexplained peritonitis. Microscopic study of the polypoid lesion showed the submucosal mass with histological and immunological features of RDD. The whole body computed tomography revealed neither lymphadenopathy nor tumor-like mass.


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