scholarly journals Parathyroid Carcinoma: An Up-to-Date Retrospective Multicentric Analysis

2020 ◽  
Vol 2020 ◽  
pp. 1-5 ◽  
Author(s):  
Francesco Quaglino ◽  
Luca Manfrino ◽  
Luca Cestino ◽  
Massimo Giusti ◽  
Enrico Mazza ◽  
...  

Parathyroid carcinoma (PC) is a rare disease responsible for about 1% of primary hyperparathyroidism (PHPT) cases. PC usually has an indolent course, tough to differentiate from the benign causes of PHPT, and the only certain diagnosis is histologic. The gold standard surgical treatment is the en bloc resection associated with the homolateral thyroid loboistmectomy. The aim of this study was to underline the main differences between PC and benign PHPT, along with gathering epidemiological knowledge relative to PC in our region. Data from the regional cancer network (Rete Oncologica del Piemonte e della Valle d’Aosta) since 2007 have been reported, including 21 patients from three hospitals (AO S. Croce e Carle of Cuneo, AOU Città della Salute of Turin, and ASL Città di Torino). The incidence of the disease, gender, age at time of diagnosis, presence of renal and bone symptoms, serum calcium and PTH levels, surgical technique performed, and percentage of recurrence were analysed. PC data were than compared with a series of patients affected by benign PHPT, referred to ASL Città di Torino, Maria Vittoria Hospital, from 2007 to 2019. A PC incidence of 0.05 cases per 100,000 inhabitants was found in our region. Benign forms occurred more frequently in females (p=0.0002), while PC equally occurred in males and females and affected younger patients (p=0.026). Serum calcium and PTH levels were significantly higher in PC patients; accordingly, typical PHPT symptoms were more frequently reported in PC than in benign PHPT. In the PC group, the en bloc resection shows a 13 times lower risk for relapse compared with all the other surgical techniques. PC is equally gender distributed, and the average patients’ age is in the fifth decade of life. It is usually functioning, with greater biochemical activity and multiple symptoms. A not-radical surgical resection is associated with a higher recurrence rate. A meticulous presurgical evaluation of PHPT patients showing PC’s evocative features is mandatory to obtain a complete disease extirpation.

2016 ◽  
Vol 24 (4) ◽  
pp. 644-651 ◽  
Author(s):  
Ziya L. Gokaslan ◽  
Patricia L. Zadnik ◽  
Daniel M. Sciubba ◽  
Niccole Germscheid ◽  
C. Rory Goodwin ◽  
...  

OBJECT A chordoma is an indolent primary spinal tumor that has devastating effects on the patient's life. These lesions are chemoresistant, resistant to conventional radiotherapy, and moderately sensitive to proton therapy; however, en bloc resection remains the preferred treatment for optimizing patient outcomes. While multiple small and largely retrospective studies have investigated the outcomes following en bloc resection of chordomas in the sacrum, there have been few large-scale studies on patients with chordomas of the mobile spine. The goal of this study was to review the outcomes of surgically treated patients with mobile spine chordomas at multiple international centers with respect to local recurrence and survival. This multiinstitutional retrospective study collected data between 1988 and 2012 about prognosis-predicting factors, including various clinical characteristics and surgical techniques for mobile spine chordoma. Tumors were classified according to the Enneking principles and analyzed in 2 treatment cohorts: Enneking-appropriate (EA) and Enneking-inappropriate (EI) cohorts. Patients were categorized as EA when the final pathological assessment of the margin matched the Enneking recommendation; otherwise, they were categorized as EI. METHODS Descriptive statistics were used to summarize the data (Student t-test, chi-square, and Fisher exact tests). Recurrence and survival data were analyzed using Kaplan-Meier survival curves, log-rank tests, and multivariate Cox proportional hazard modeling. RESULTS A total of 166 patients (55 female and 111 male patients) with mobile spine chordoma were included. The median patient follow-up was 2.6 years (range 1 day to 22.5 years). Fifty-eight (41%) patients were EA and 84 (59%) patients were EI. The type of biopsy (p < 0.001), spinal location (p = 0.018), and if the patient received adjuvant therapy (p < 0.001) were significantly different between the 2 cohorts. Overall, 58 (35%) patients developed local recurrence and 57 (34%) patients died. Median survival was 7.0 years postoperative: 8.4 years postoperative for EA patients and 6.4 years postoperative for EI patients (p = 0.023). The multivariate analysis showed that the EI cohort was significantly associated with an increased risk of local recurrence in comparison with the EA cohort (HR 7.02; 95% CI 2.96–16.6; p < 0.001), although no significant difference in survival was observed. CONCLUSIONS EA resection plays a major role in decreasing the risk for local recurrence in patients with chordoma of the mobile spine.


2006 ◽  
Vol 63 (8) ◽  
pp. 765-769
Author(s):  
Aleksandar Filipovic ◽  
Ivan Paunovic ◽  
Dragutin Savjak ◽  
Tamara Zivkovic

Background. Parathyroid carcinoma is the least frequent malignancy among endocrine tumors. In the most reported series of patients with primary hyperparathyroidism the incidence of carcinoma is less than 1%. Recognition by a surgeon that the parathyroid tumor is malignant, and the performance of an adequate en bloc removal of primary lesion, with histologic diagnosis offer the best treatment of a patient with this unusual malignancy. Case report. We reported a 30-year-old patient with parathyroid carcinoma, primary hyperparathyroidism, and recurrent nephrocalcinosis. Marked hypercalcemia, low serum phosphorus, and substantial elevation of serum parathyroid hormone indicated a diagnosis of primary hiperparathyroidism. General symptoms were anorexia, muscle weakness, back pain and depression. Ultrasonography done before the surgery revealed a 2 cm upper left parathyroid gland with solid and cystic areas. The neck exploration was done with en block resection of the tumor. A histopathological evaluation confirmed the diagnosis of parathyroid carcinoma. Over more than a three-year-follow-up, the patient had no evidence of the disease recurrence and his serum PTH and calcium levels remained within the normal. Conclusion. Parathyroid carcinoma is a rare cause of primary hyperparathyroidism. Preoperative diagnosis remains a challenge. Radical en bloc resection of the tumor is the treatment of choice for this malignancy.


2021 ◽  
Author(s):  
Amanda N Sacino ◽  
Sutipat Pairojboriboon ◽  
Ian Suk ◽  
Daniel Lubelski ◽  
Robin Yang ◽  
...  

Abstract BACKGROUND AND IMPORTANCE En bloc resection of sacral tumors is the most effective treatment to help prevent recurrence. Sacrectomy, however, can be destabilizing, depending on the extent of resection. Various surgical techniques for improving stability and enabling early ambulation have been proposed. CLINICAL PRESENTATION Here, we report a case in which we use PMMA (poly[methyl methacrylate]) to augment pelvic instrumentation to improve mechanical stability after sacrectomy for en bloc resection of a solitary fibrous tumor. CONCLUSION We highlight the use of sacroplasty augmentation of pelvic ring reconstruction to provide biomechanical stability without the need for fusion of any mobile spine segments, which allowed for early patient ambulation and no appreciable loss of range of motion or mobility.


1990 ◽  
Vol 50 (1) ◽  
pp. 138-140 ◽  
Author(s):  
Joe B. Putnam ◽  
Stimson P. Schantz ◽  
William C. Pugh ◽  
Robert C. Hickey ◽  
Naguib A. Samaan ◽  
...  

2021 ◽  
Vol 11 (3) ◽  
pp. 88-94
Author(s):  
Andrei I Gritsiuta

Primary benign tumors of the sternum are an exceedingly rare entity. Surgical techniques regarding intervention for these lesions are not clearly defined in the literature given their scarcity. Operative techniques include en-bloc resection of the tumor, and this has proven to be successful in preventing local recurrence despite benign nature of the lesion. Given the often extensive defect created by the excision, reconstruction is frequently necessary; depending on the size of the defect, either autologous bone grafting or the use of synthetic materials may be indicated. This study serves to present two cases of rare primary benign tumors of the sternum, giant cell tumors and osteoma spongiosum and to summarize the available literature. We present a review of the literature of 17sternal giant cell tumor cases reported so far including our patient and unique case of osteoma spongiosum of the sternum, that discusses their surgical management, as well as reconstructive techniques that provided an excellent clinical result and a lack of recurrence on long term follow-up.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Tina Y Hu ◽  
Loren Berman ◽  
William J Parkes ◽  
Jeanne M Franzone ◽  
Tejal Mody ◽  
...  

Abstract BACKGROUND: Parathyroid carcinoma (PC) is exceptionally rare in children and its clinical features are poorly understood. Available reports have identified that very high serum calcium and parathyroid hormone levels would be expected in this disease. Definitive therapy for PC requires en bloc resection of the parathyroid tumor to reduce the risk of disease recurrence. Unfortunately, there are very few treatment options for metastatic PC and patients with recurrent PC have a high risk of mortality related to their disease. Because of the rarity of the condition, there are very limited data including pediatric patients. Case: A previously healthy 13-year-old girl initially presented to primary care with bilateral foot and ankle pain in the context of acute and progressive genu valgum, referred to the orthopedic clinic. Lab evaluation prompted by imaging demonstrated severe hypercalcemia (15.6 mg/dL) and hyperphosphatasia (Alkaline phosphatase 2056 units/L). She was urgently referred to our clinic where we found her to have a firm right sided neck mass within the thyroid. Lab testing at that time confirmed profound hyperparathyroidism (PTH 2970 pg/mL). Neck ultrasound confirmed the presence of a 5.8 cm hypoechoic lesion adjacent to the right lower lobe of the thyroid. The patient received IV fluid resuscitation before undergoing urgent parathyroid surgery. The parathyroid lesion was grossly adherent to the thyroid gland and she received en bloc resection of the tumor and right lobe of the thyroid. Post-operatively, PTH rapidly declined to &lt;3.4 and she developed a prolonged hypocalcemic phase. PTH function improved, but she continued to require high doses of calcium carbonate to address hungry bone syndrome over subsequent weeks. Comprehensive evaluation included testing for genetic mutations, deletions, and duplications in CASR, CDC73, CDKN1B, MEN1 and RET, all of which were negative. Extensive genetic testing of the parathyroid tumor did not identify any specific mutations which have been previously associated with PC. Six months post-operatively, her labs have vastly improved, although she has clinical evidence of PTH resistance and her alkaline phosphatase has not yet normalized. Neck ultrasound, F-18 FDG PET/MRI imaging and Sestamibi scans show no evidence of persistent disease. Conclusions: While parathyroid carcinoma is extremely rare in pediatric patients, the diagnosis must be considered in cases of hypercalcemia with marked elevations in PTH and palpable neck mass. Although rarely described, bone changes related to PC may be dramatic and may result in lasting morbidity - including genu valgum requiring surgical intervention as well as prolonged hungry bone syndrome and metabolic bone disease. Genu valgum is a unique pediatric feature of this disease because this may only evolve in individuals whose physes are open.


2021 ◽  
Vol 24 (1) ◽  
pp. 28-33
Author(s):  
Anjan Singh Karki ◽  
Dipendra Kumar Shresha ◽  
Gopal Sedain ◽  
Sushil Krishna Shilpakar

Scalp arteriovenous malformation (AVM) accounts for only 8.1% of all AVM cases. These lesions are made up of an abnormal fistulous tangle of tortuous dysmorphic network of vessels “vascular nidus” directly connecting between the feeding arteries and draining veins, without capillary connection, located within the incision subcutaneous layer. With time, these congenital lesions may evolve and enlarge and clinically manifest with variable features. The only effective method of preventing evolution of these malformations is to exclude the lesion completely from the circulation. Involvement of the orbit and face may cause severe facial disfigurement, proptosis, visual obstruction and even facial palsy. These lesions may be complicated by ulceration, infection and profuse bleeding. We present a rare case of 40-year-old female with a recurrent congenital scalp-orbital AVM causing proptosis, visual obstruction on the right eye with severe facial disfigurement. The lesion was successfully excised with acceptable cosmesis and removal of the visual obstruction. A brief literature review, imaging findings and the surgical techniques have been presented.


2015 ◽  
Vol 22 (2) ◽  
pp. 139-150 ◽  
Author(s):  
Michelle J. Clarke ◽  
Patricia L. Zadnik ◽  
Mari L. Groves ◽  
Daniel M. Sciubba ◽  
Timothy F. Witham ◽  
...  

OBJECT Recently, aggressive surgical techniques and a push toward en bloc resections of certain tumors have resulted in a need for creative spinal column reconstruction. Iatrogenic instability following these resections requires a thoughtful approach to adequately transfer load-bearing forces from the skull and upper cervical spine to the subaxial spine. METHODS The authors present a series of 7 cases in which lateral mass reconstruction with a cage or fibular strut graft was used to provide load-bearing support, including 1 case of bilateral cage placement. RESULTS The authors discuss the surgical nuances of en bloc resection of high cervical tumors and explain their technique for lateral mass cage placement. Additionally, they provide their rationale for the use of these constructs throughout the craniocervical junction and subaxial spine. CONCLUSIONS Lateral mass reconstruction provides a potential alternative or adjuvant method of restoring the load-bearing capabilities of the cervical spine.


2021 ◽  
Vol 104 (6) ◽  
pp. 1033-1038

Background: Parathyroid carcinoma (PC) is a rare malignancy that is difficult to diagnose preoperatively, and when it is suspected from clinical features and laboratory results, en-bloc resection is performed. Objective: To retrospectively review the prevalence, clinical presentations, laboratory findings, management modalities, and treatment outcomes of PC cases in the present study tertiary referral hospital. Materials and Methods: Patients with PC that attended the present center between 2008 and 2019 were included. Demographic data, clinical presentations, preoperative laboratory results, management, and outcomes were retrospectively reviewed. Results: Ten of 102 cases (9.8%) of the primary hyperparathyroidism were diagnosed as PC. Five were male (50%), and the mean age at diagnosis was 47.90±15.50 years. All PC patients presented with renal or bony manifestations, and five out of nine (45.5%) had palpable neck mass. Mean preoperative parathyroid hormone and serum calcium were 1,688.56±858.84 pg/mL and 15.30±3.27 mg/dL, respectively. In eight of these PC cases (80%), en-bloc resection was performed, and six patients underwent postoperative radiation treatment. Two patients died due to uncontrolled hypercalcemia with distant metastasis at 5.5- and 8-years after diagnosis. Conclusion: The prevalence of PC among primary hyperparathyroidism is as high in Thailand as in other Asian countries. En-bloc resection is performed when clinical presentations and laboratory results are suggestive of PC. Postoperative radiation is considered in selected cases. Intractable hypercalcemia is the main cause of death. Keyword: Parathyroid cancer; Parathyroid carcinoma; Primary hyperparathyroidism; Hypercalcemia; Parathyroid carcinoma case series


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