Paraneoplastic syndromes in patients with nasopharyngeal cancer
Section snippets
Nasopharyngeal carcinoma
Nasopharyngeal carcinoma (NPC) is relatively uncommon in Western countries, but is 10–50 times more common in the Far East, and is endemic in the Southern parts of China with the yearly incidence rate varying between 15 per 100,000 and 50 per 100,000 [1]. In Hong Kong, NPC is the third most common malignancy in male and the fifth in female patients with an incidence of 27.5 and 11.2 per 100,000, respectively [2]. The particularly high frequency of malignancies of the nasopharynx in the Far East
Paraneoplastic syndromes
Paraneoplastic syndromes (PNS) represent the clinical manifestation of the remote and indirect effects produced by tumor metabolites or other products. Paraneoplastic effects are not directly mediated by the invasion of normal tissue, or by the disruption of normal function of the involved organ, or by distant metastases [6].
PNS occur in 1–7.4% of all cancer patients [7]. Different terms such as paraneoplastic effects, remote effects, paraneoplastic events, non-metastatic syndromes,
Paraneoplastic cutaneous or dermatologic syndromes
First described in 1916 by Stertz [12], the association of connective tissue diseases, particularly dermatomyositis (DM) and scleroderma, with a variety of malignancies has been reported frequently in recent decades. DM seems to have a robust association with malignancy, with studies showing a frequency of cancer of 15–34% in some medical centers. In the upper aerodigestive tract, this phenomenon is seen most commonly with NPC [13]. DM is a severe systemic disorder characterized by typical
Paraneoplastic endocrine syndromes
Patients affected by NPC usually consult the endocrinologist when they develop hypopituitarism as a consequence of radiotherapy. The majority of patients present with local symptoms due to the tumor and it is rare for patients with occult NPC to present initially with endocrine manifestations.
Paraneoplastic endocrine syndromes result from aberrant production of protein hormones, hormone precursors, or hormone-like substances by tumors. Ectopic or inappropriate hormone secretion causing
Paraneoplastic hematologic syndromes
Tumor fever and leukemoid reaction are associated with NPC as PNS. They are usually observed in patients with metastatic disease. Tumor fever is frequently associated with other PNS, of which leukemoid reaction is the most common. These remote effects are not produced as a direct effect of the tumor or its metastasis.
A single case of hematologic syndrome was reported in 1985 by Maalej et al. [9]. In 1993, Cvitkovic et al. [40] reported a cohort of 255 patients affected by undifferentiated NPC.
Paraneoplastic osteoarticular or rheumatologic syndromes
The real incidence of paraneoplastic osteoarticular syndromes is difficult to determine, because NPC can be associated with finger clubbing as a part of a paraneoplastic condition or as an external manifestation of occult pulmonary metastases [44]. Many “false-paraneoplastic” osteoarticular syndromes are reported in the head and neck literature, in which symptoms were related to direct disruption of the normal function of the lungs by distant metastases. Indeed, the most common site of distant
Paraneoplastic neurologic syndromes
Paraneoplastic neurologic syndromes are believed to be autoimmune and due to activation of the immune system to destroy distant tumors. The neural tissues are damaged by immune-mediated inflammation as they express onconeural proteins coexpressed by tumors [53]. Both the central and peripheral nervous systems can be affected. Sensory neuropathy is a classical paraneoplastic neurologic syndrome affecting the peripheral nervous systems associated with solid tumors, mostly common small cell lung
Paraneoplastic ocular syndromes
The most common paraneoplastic ocular syndromes in head and neck cancer are cancer-associated retinopathy and melanoma-associated retinopathy [56]. These have been occasionally reported in association with cancer of the larynx and hypopharynx.
NPC is commonly manifest as a neck mass, nasal obstruction, nasal bleeding, and occasionally hearing loss. NPC typically presents to ophthalmologists with one or more cranial nerve palsies. The fifth and sixth nerves are the most commonly affected. These
Conclusions
PNS may precede the clinical manifestation of a persistent or recurrent tumor or of asymptomatic metastases [59].
In the literature PNS are mostly described in case reports or review articles. Excluding false-paraneoplastic cases, more than 260 NPC have been reported with PNS (Table 1).
The exact incidence of PNS in association with head and neck cancer is not known, but it is surely underestimated, because many cases are not reported in the literature or they are not well recognized. The
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Cited by (22)
Diagnostic challenges in a case of an isolated third nerve palsy
2020, American Journal of Ophthalmology Case ReportsCitation Excerpt :A similar case was reported by Beckam et al., in 2010 which states that the extent of the tumor on MRI fails to indicate why the third cranial nerve was the only affected cranial nerve.12 More than 260 cases of NPCs associated with paraneoplastic syndrome (PNS) affecting various organ systems have been reported.13 PNS occurs due to immune cross-reactivity between tumor cells and normal tissue.
Nasopharyngeal cancers, an overview
2014, Bulletin du CancerCutaneous alerts in systemic malignancy: Part 1
2013, Actas Dermo-SifiliograficasCitation Excerpt :The former is usually hereditary and progresses slowly, while the latter—the paraneoplastic form—is associated with cardiopulmonary disorders. In 80% of cases, the paraneoplastic presentation is associated with lung cancer, metastases to the lung, nasopharyngeal carcinoma, and other less common cancers.19,25,29,66,67 Vascular skin manifestations in patients with underlying malignancies are quite nonspecific.5
Nasopharyngeal carcinoma in dermatomyositis patients: A 10-year retrospective review in Hospital Selayang, Malaysia
2012, Reports of Practical Oncology and RadiotherapyCitation Excerpt :Dermatomyositis is defined as an idiopathic inflammatory myopathy with clinically distinctive cutaneous manifestations.1 However, when the symptoms are associated with malignancies, the condition is known as paraneoplastic syndrome which represents the clinical manifestation of the remote and indirect effects produced by tumor metabolites or other products.2 Dermatomyositis is highly associated with various types of malignancies, namely carcinoma of nasopharynx, breast, lung, colorectal, uterus and non-Hodgkin lymphoma.3
Paraneoplastic dermatomyositis and nasopharyngeal carcinoma: Diagnosis, treatment and prognosis
2011, Annales Francaises d'Oto-Rhino-Laryngologie et de Pathologie Cervico-FacialeParaneoplastic dermatomyositis accompanying nasopharyngeal carcinoma: Diagnosis, treatment and prognosis
2011, European Annals of Otorhinolaryngology, Head and Neck DiseasesCitation Excerpt :NPC accounts for less than 1% of all malignancies and affects approximately one person in 100,000 in North America and Western Europe, but is more frequent in Asia and North Africa, affecting five to nine per 100,000 of the population [4]. It is particularly frequent in southern China, totaling almost one-third of all malignancies treated, according to Hu et al. [2] It is characterized by the frequency of an undifferentiated pathological subtype, its relationship to Epstein-Barr virus with viral DNA detectable in tumor cells, a high metastatic potential and frequent association with paraneoplastic syndromes such as fever, clubbing osteoarthropathy and leukemoid reactions [5]. DM is an idiopathic inflammatory myopathy with prominent and often characteristic cutaneous manifestations.