Parastruma maligna aberrata

Quervain, F. de

doi:10.1007/bf02819737

Cited by 60 publications

(20 citation statements)

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“…[1][2][3][4] de Quevain 5 described this entity first in 1904 in a patient who presented with a nonfunctioning lesion. Subsequently, in 1933, Sainton and Millot 6 described the first functioning parathyroid carcinoma.…”

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confidence: 99%

Parathyroid carcinoma: Evaluation and interdisciplinary management

Clayman

González

El‐Naggar

et al. 2004

Cancer

117

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BACKGROUNDA review of recent reports found a distinct clinical behavior pattern in the rare clinical entity of parathyroid carcinoma, although to the authors' knowledge information on oncogenetic changes, prognostic factors, and the potential benefits of adjuvant therapy remain fragmented and scarce. In this report, a composite review of the literature and The University of Texas M. D. Anderson Cancer Center (M. D. Anderson) experience are presented using the presentation of a patient to illustrate critical issues in the evaluation and interdisciplinary management of patients who are afflicted with this disease.METHODSThe current study reflects a retrospective case review of patients who were diagnosed with parathyroid carcinoma, treated, and followed at M. D. Anderson from 1983 to 2002. To assure standardization of pathologic diagnosis as well as evaluations and interdisciplinary management, the investigators reviewed all cases using predetermined criteria within their specialties.RESULTSIt is interesting to note that M. D. Anderson data showed classic pathologic features that were not always present in all parathyroid carcinomas (at most, some features were noted in 37% of patients). Other results of interest indicated local recurrence rates that appeared lower if adjuvant radiation was applied after initial surgery, independent of the type of surgery or disease stage. In the authors' experience, 70% of patient's tumors exhibited local invasion, although their 5‐year survival rate of 85% was consistent with that reported previously, and their 10‐year survival rate was somewhat higher at 77%.CONCLUSIONSParathyroid carcinoma is a rare clinical entity that requires interdisciplinary evaluation and management. Comprehensive surgical excision of parathyroid carcinomas with verification of normalization of intraoperative parathyroid hormone levels should be sought. Cancer 2004;100:900–5. © 2004 American Cancer Society.

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confidence: 99%

Parathyroid carcinoma: Evaluation and interdisciplinary management

Clayman

González

El‐Naggar

et al. 2004

Cancer

117

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“…The only curative treatment is en bloc resection (Flye and Brennan, 1981;Wynne et al, 1992). The few reports on nonproducing parathyroid carcinoma suggest a different disease with a more aggressive behavior (Table 1) (Aldinger et al, 1982;de Quervain, 1909;Guy, 1929;Hall and Chaf n, 1934;McQuillain, 1938;Mendiola, 1942;Merlano et al, 1985;Ordonez et al, 1983;Price and Mowat, 1932;Roffo and Landivar, 1914;Sieracki and Horn, 1960;Toland, 1931). The etiology of parathyroid carcinoma is unknown.…”

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confidence: 99%

Painful ophthalmoplegia from metastatic nonproducing parathyroid carcinoma: Case study and review of the literature

Eurelings¹,

Frijns²,

Jeurissen³

2002

Neuro-Oncol

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Parathyroid carcinoma is an uncommon malignancy. Of the fewer than 400 cases reported, most have been cases of producing parathyroid carcinoma with accompanying hypercalcemia. Only 13 patients with nonproducing parathyroid carcinoma have been described. Nine of these 13 patients had metastatic disease. We report a patient with i.c. metastasis. Distal metastases of producing parathyroid carcinoma are treated surgically to prolong survival and prevent complications of hyperparathyroidism and hypercalcemia. One half of the patients with producing parathyroid carcinoma die within 5 years, mostly because of the complications of hypercalcemia. Nonproducing parathyroid carcinoma compares unfavorably with producing parathyroid carcinoma in terms of tumor progression and prognosis. Few data on choice of therapy in nonproducing parathyroid carcinoma are available. We treated our patient with a combination of radiotherapy and chemotherapy. Treatment was followed by an unexpectedly prolonged survival of 31 months after diagnosis of metastatic disease. Neuro-Oncology 4, 44-48, 2002 (Posted to Neuro-Oncology [serial online], Doc. 01-023, October 3, 2001 W e report a patient who had nonproducing parathyroid carcinoma with painful ophthalmoplegia from i.c. metastasis. We treated this patient with cranial radiotherapy (5 3 400 cGy in 1 week) followed by 3 cycles of chemotherapy once every 3 weeks, with a combination of cisplatin (100 mg/m 2 on day 1) and etoposide (120 mg/m 2 on days 1, 2, and 3). In view of the extreme rarity of the tumor, clinical trials to test different treatment modalities are not feasible. Therefore, the development of future treatment strategies depends on anecdotal reports. Case StudyA 45-year-old woman was treated for Graves' disease at another hospital. After 1 year she developed problems swallowing and speaking. Because of suspected thyroid carcinoma, a hemithyroidectomy was performed. Pathological examination showed a nonproducing parathyroid carcinoma ( Fig. 1) (Schantz and Castleman, 1973;Vetto et al., 1993). Because the tumor was not radically removed, she received radiotherapy (4000 cGy at once followed by 6000 cGy in fractions in 4 weeks at the tumor site). One year later, she presented with headache, vomiting, double vision, and a painful left eye. At examination, ptosis and abduction paresis of the left eye were found. A cerebral MRI scan showed an enhancing lesion in and around the cavernous sinus ( Fig. 2A and 2B). The patient was treated with high-dose prednisolone, up to 80 mg per day, because of a tentative diagnosis of Tolosa Hunt syndrome (Yousem et al., 1990). Because the symptoms and signs did not subside, lymphoma or metastatic tumor were suspected. On admission, she had a complete bilateral ophthalmoplegia with ptosis and absent pupillary light re exes. Vision was unimpaired. Sensation was decreased bilaterally in the area of the rst branch of the trigeminal nerve. No abnormalities were found during routine laboratory tests, which included tests for thyroid stimulating hormone, T4...

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“…Il paziente era un uomo di 68 anni che si presentava con una grande massa al collo e che morì subito dopo presentando recidiva locale e metastasi polmonari. Nella descrizione di tale paziente, De Quervain non menzionò alcun segno o sintomo di ipercalcemia [5]. I carcinomi paratiroidei non funzionanti sono estremamente rari, 26 casi dal 1929 [6], e si presentano con segni e sintomi della crescita tumorale e dell'invasione locale (massa al collo visibile, raucedine, disfagia).…”

Section: Presentazione Clinicaunclassified

Il carcinoma delle paratiroidi

Salcuni

Scillitani

2015

L'Endocrinologo

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Sommario Il carcinoma (K) delle paratiroidi è una patologia maligna rara che può presentarsi in modo sporadico o essere parte di una sindrome genetica. Il quadro clinico è generalmente caratterizzato dai segni e sintomi dell'ipercalcemia e, più raramente, dall'effetto massa della lesione. In genere è funzionante, anche se sono state descritte forme non secernenti. Distinguere in base alle caratteristiche cliniche e biochimiche tra lesione maligna e lesione benigna non è possibile, per cui quando viene posto il sospetto, il trattamento deve essere l'intervento chirurgico di asportazione en bloc della lesione e dell'emitiroide ipsilaterale con consensuale linfoadenectomia del comparto centrale. Questo tipo di intervento, infatti, se effettuato alla diagnosi, dà la più alta probabilità di guarigione. I controlli biochimici e strumentali devono essere effettuati con regolarità e protratti nel tempo per l'elevato rischio, anche dopo molti anni, di recidiva locale e di metastasi a distanza. Questo articolo si focalizzerà su alcuni aspetti clinici e patogenetici della malattia; inoltre, ci darà delle informazioni sul suo trattamento e follow-up.Parole chiave Ipercalcemia · Iperparatiroidismo primitivo · Carcinoma delle paratiroidi · CDC73 · Cinacalcet Proposto da Emanuela Arvat.Materiale elettronico supplementare La versione elettronica di questo articolo ( EpidemiologiaLa prevalenza e l'incidenza del carcinoma paratiroideo sono molto basse, tanto da essere riportate come percentuale di iperparatiroidismo primitivo (PHPT). La più ampia serie di pazienti con carcinoma (K) paratiroideo deriva dal National Cancer Database.Il K paratiroideo solitamente insorge intorno alla 4 a -5 a decade di vita, sebbene siano descritti anche casi in età pediatrica, e non vi è differenza di frequenza tra i due sessi.Nella forma sporadica la prevalenza nei soggetti con PHPT risulta essere inferiore all'1%, con l'eccezione della popolazione giapponese, dove è stata riportata un'incidenza pari a circa il 5% dei soggetti con PHPT [1]. Diagnosi Patogenesi e biologia molecolareLe cause del carcinoma paratiroideo non sono ancora note. Sono state descritte associazioni con particolari sindromi cliniche e identificate mutazioni in alcuni geni. Un incremento dell'incidenza di K paratiroideo sembra esser presente nei soggetti con insufficienza renale in fase terminale e nei soggetti con una pregressa irradiazione al collo. In realtà, per quanto riguarda l'irradiazione l'associazione risulta piuttosto debole, se paragonata a quanto descritto nella forma benigna. Sono stati descritti alcuni casi di carcinoma paratiroideo in soggetti con Neoplasia Endocrina Multipla di tipo 1 (MEN1) e uno solo in un paziente con MEN2A. Sembra queste siano associazioni casuali e non esista ad oggi un'ipotesi patogenetica che possa giustificare l'insorgenza del carcinoma in queste patologie familiari [1].

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Parastruma maligna aberrata

Cited by 60 publications

References 6 publications

Parathyroid carcinoma: Evaluation and interdisciplinary management

Parathyroid carcinoma: Evaluation and interdisciplinary management

Painful ophthalmoplegia from metastatic nonproducing parathyroid carcinoma: Case study and review of the literature

Il carcinoma delle paratiroidi

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