scholarly journals Phosphaturie mesenchymal tumour, 'non-phosphaturic' variant: a case report and review of the literature

2021 ◽  
Vol 20 (3) ◽  
Author(s):  
Reubina Wadee ◽  
Zwelithini Linda ◽  
Abdullah Ismail

ABSTRACT BACKGROUND: Phosphaturic mesenchymal tumours are uncommon neoplasms, usually associated with tumour-induced osteomalacia, that occur in middle-aged adults but have been reported at the extremes of age. The 'non-phosphaturic' variant is even rarer METHODS: Herein, we describe the non-phosphaturic variant in a 12-year-old male who presented to the Department of Orthopaedic Surgery with a six-month history of painful progressive swelling of his right forearm in the absence of trauma. He had no other significant symptoms RESULTS: The patient had normal serum calcium and phosphate levels on biochemical analysis. An inci-sional biopsy was performed and a histopathological diagnosis of a phosphaturic mesenchymal tumour, non-phosphaturic variant, was rendered. Six weeks later, he underwent surgical excision of a 15x15 cm soft tissue mass from his right forearm. He had an uneventful postoperative period and was discharged. He has been followed up at the outpatient department and has been free of tumour recurrence for over 18 months since his surgery with no evidence of osteomalacia and no other tumours CONCLUSION: Phosphaturic mesenchymal tumours are rare mesenchymal neoplasms and their microscopically identical counterpart without phosphaturia, known as the 'non-phosphaturic' variant, is even more uncommon and may prove a greater diagnostic challenge. While the diagnosis may be confirmed by hypophosphataemia and phosphaturia secondary to the paraneoplastic phenomenon of tumour-induced osteomalacia, there may be instances, such as with our patient, where tumour-induced osteomalacia is absent. This case underscores the importance of clinicopathological correlation together with a wide differential diagnosis required to arrive at a correct diagnosis to facilitate appropriate patient management Level of evidence: Level 5 Keywords: phosphaturic mesenchymal tumours, non-phosphaturic variant

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
K. Bokhari ◽  
M. S. Hameed ◽  
M. Ajmal ◽  
Rafi A. Togoo

Background. Osteoblastoma is a rare benign tumor. This tumor is characterized by osteoid and bone formation with the presence of numerous osteoblasts. The lesion is more frequently seen in long bones and rarely involves maxilla and mandible. Due to its clinical and histological similarity with other bone tumors such as osteoid osteoma and fibro-osseous lesions, osteoblastoma presents a diagnostic dilemma.Case Report. Very few cases of osteoblastomas involving maxillofacial region have been reported in the literature. This case report involves osteoblastoma involving right maxilla in an 18-year-old male patient. Following detailed clinical examination, radiological interpretation, and histopathological diagnosis, surgical excision was performed. The patient was followed up for a period of 3 years and was disease free.Summary and Conclusion. Benign osteoblastoma involving jaw bones is a rare tumor. There is a close resemblance of this tumor with other lesions such as fibro-osseous lesions and odontogenic tumors and thus faces a diagnostic challenge. Surgical excision with a long-term follow-up gives good prognosis to this lesion—Benign Osteoblastoma.


2010 ◽  
Vol 58 (3) ◽  
pp. 331-340 ◽  
Author(s):  
Csaba Jakab ◽  
Miklós Rusvai ◽  
Nóra Biró ◽  
Zoltán Szabó ◽  
Péter Gálfi ◽  
...  

A 5-year-old female degu ( Octodon degus ) showed the clinical sign of metrorrhagia. During ovariohysterectomy a circumscribed tumoural lesion was found in the right uterine horn. The histopathological diagnosis of this soft tissue mass was primary benign cavernous angioleiomyoma of the uterus. During immunohistochemical analysis the neoplastic endothelial cells of this mixed mesenchymal tumour showed strong membrane positivity for the endothelial marker claudin-5 but were negative for CD31 (another endothelial marker). The endothelial cells of the internal positive control tissues such as intact peritumoural vessels were positive for claudin-5 but negative for the CD31 endothelial marker. As it has been described also in other species, it seems that claudin-5 is a better endothelial marker than CD31 for the detection of normal and neoplastic endothelial cells in different tissues of degus.


2014 ◽  
Vol 7 (2) ◽  
pp. 87-89 ◽  
Author(s):  
Neelam Wadhwa ◽  
PP Singh ◽  
Vipin Arora ◽  
Pankaj Verma ◽  
Khyati Bhatia

ABSTRACT A rare case of sinonasal paraganglioma is described. A 40-year-old female patient presented with 2 years history of unilateral nasal obstruction and bleeding. CT scan demonstrated an expansile enhancing mass involving bilateral ethmoids, right nasal cavity and right maxillary sinus. Histopathological diagnosis was neuroendocrine tumor with possibility of paraganglioma. A subtotal maxillectomy with excision of mass performed. Primary nonchromaffin paraganglioma of nose and paranasal sinus is a very rare tumor, these lesion pose diagnostic challenge to clinicians and pathologist. Only twenty five cases are reported in review of literature. Surgical excision is the mainstay of treatment. How to cite this article Arora V, Verma P, Singh PP, Wadhwa N, Bhatia K. Sinonasal Paraganglioma: A Case Report and Review of Literature. Clin Rhinol An Int J 2014;7(2):87-89.


2021 ◽  
Author(s):  
Ioana Anca Stefanopol ◽  
Dumitru Marius Danila ◽  
Georgiana Bianca Constantin ◽  
Liliana Baroiu

Abstract BackgroundParaovarian cysts (POCs) are rarely seen among pediatric female patients. The diagnosis still represents a challenge. Giant POCs are exceedingly rare and only few cases have been reported in the literature.Case presentationA 17-year-old girl from the rural area presented with abdominal pain, pollakiuria and a giant abdomino-pelvic mass. Ultrasound and computed tomography revealed a 14cm x 24cm x 30cm anechoic unilocular cyst which raised the suspicion of a mesenteric or ovarian cyst. The correct diagnosis of left POC was established intraoperatory. Cystectomy was performed. Histopathological diagnosis was serous cystadenoma.DiscussionsPOCs become symptomatic when reach large sizes or complicate, the most feared complication being ovary torsion. Imagistic investigations can establish the diagnosis of cyst, but the cyst’s origin often remains an intraoperatory discovery. Surgical excision is necessary to decrease the risk of complications, and adnexal preservation is essential in pediatric patients. Even for pediatric female patients POC should be considered in the differential diagnosis of cystic abdominal masses.


2016 ◽  
Vol 2016 ◽  
pp. 1-6
Author(s):  
Xiangshui Sun ◽  
Yue Lou ◽  
Xiaodong Wang

Iliac bone destruction in children is uncommon and presents various imaging features. Correct diagnosis based on clinical and imaging features is difficult. This research aimed to retrospectively explore the clinical features, imaging, and histopathological diagnosis of children with iliac bone destruction. A total of 22 children with iliac bone destruction were enrolled in this retrospective analysis from two children’s hospitals during July 2007 to April 2015. Clinical features, imaging, and histopathological findings were analysed. The mode of iliac bone destruction, lesion structure, and the relationship between the range of soft tissue mass and cortical destruction were determined based on imaging data. The data were analysed using descriptive methods. Of the iliac bone destruction cases, eight cases were neuroblastoma iliac bone metastasis, seven cases were bone eosinophilic granuloma, two cases were Ewing’s sarcoma, two cases were osteomyelitis, one case was bone cyst, one case was bone fibrous dysplasia, and one case was non-Hodgkin’s lymphoma. Iliac bone destruction varies widely in children. Metastatic neuroblastoma and eosinophilic granuloma are the most commonly involved childhood tumours.


2019 ◽  
Vol 5 (2) ◽  
pp. 205511691985787
Author(s):  
Filippo Torrigiani ◽  
Giorgio Romanelli ◽  
Paola Roccabianca ◽  
Elisabetta Treggiari

Case summary A 7-year old male neutered domestic shorthair cat was presented with a 2 month history of a slow-growing mass on the right zygomatic area. A CT scan revealed a soft tissue mass in the right zygomatic region with no alterations of the underlying bone and features of local invasiveness. Cytology was suggestive of a mesenchymal tumour and histopathology from an incisional biopsy was consistent with a soft tissue sarcoma (STS). The cat was treated with neoadjuvant intravenous doxorubicin chemotherapy at a dose of 25 mg/m2, every two weeks. The patient experienced a partial response and underwent surgical excision of the tumour. Doxorubicin was continued as an adjuvant treatment for three further chemotherapy sessions, at a dose of 25 mg/m2 every 21 days. Local tumour recurrence was detected on clinical examination and cytologically confirmed 259 days following surgery. Relevance and novel information Treatment with neoadjuvant doxorubicin can be considered in cases of inoperable STSs in order to cytoreduce the tumour and improve the chances of achieving complete surgical margins. The role of adjuvant chemotherapy in this setting remains unclear.


2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Krithika Chandrasekar Lakshmi ◽  
Sathasivasubramanian Sankarapandiyan ◽  
Venkata Sai Pulivadula Mohanarangam

Intramuscular hemangiomas are unique vascular tumors which are benign in nature, most commonly occurring in the trunk and extremities. When present in head and neck, they most frequently involve the masseter and trapezius muscles, accounting for less than 1% of all hemangiomas. Most of these lesions present with pain and discomfort and some patients may demonstrate progressive enlargement. Due to their infrequency, deep location, and unfamiliar presentation, these lesions are seldom correctly diagnosed clinically. Our report is a clinically misdiagnosed case of a painful soft tissue mass in the right side masseteric region of a 23-year-old female patient, confirmed as intramuscular hemangioma based on imaging studies and histopathologic examination, treated by surgical excision which had no recurrence after a 3-year followup.


2015 ◽  
Vol 2015 ◽  
pp. 1-2
Author(s):  
M. Davari ◽  
B. W. Lamb ◽  
S. Chowdhury ◽  
C. Jameson ◽  
J. D. Kelly ◽  
...  

Aggressive angiomyxoma is a rare mesenchymal tumour predominantly affecting the female pelvis and perineum but has also been described in males. This tumour can often present a diagnostic challenge and has a propensity for local recurrence after surgical excision. We present an unusual case of aggressive angiomyxoma arising from the bladder of a female patient which required local excision and Mitrofanoff formation.


Author(s):  
Andrzej Cieszanowski ◽  
Agnieszka Anysz-Grodzicka ◽  
Joanna Podgorska ◽  
Beata Jagielska ◽  
Jakub Pałucki

<P>Background: Primary Hepatic Epithelioid Haemangioendothelioma (HEHE) and Primary Hepatic Angiosarcoma (PHA) are rare mesenchymal tumours with different malignant potential. Whereas HEHE demonstrates low to intermediate malignant potential, PHA is an aggressive malignancy with poor prognosis. The knowledge of typical imaging features of these lesions may facilitate correct diagnosis; however, the ultimate diagnosis of HEHE and PHA is based on histopathologic examination. </P><P> Discussion: The most typical findings helpful in diagnosing HEHE are: Presence of multiple, confluent nodules located at the liver periphery (in young to middle-aged woman), retraction of the liver capsule, marked hyperintensity on T2-weighted images, “target-sign” appearance, progressive centripetal contrast enhancement, and relatively high Apparent Diffusion Coefficient (ADC) values. More than &#8805;50% of nodules are hyper- or isointense on Hepatobiliary Phase (HBP) images. Conclusion: The imaging features suggestive of PHA are: Occurrence of metastases (lungs, spleen) at the time of diagnosis, presence of a large dominant mass with smaller satellites, heterogeneity and areas of haemorrhage in a dominant mass, progressive contrast enhancement, slightly elevated ADC values as compared to other malignant liver tumours.</P>


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