Paraneoplastic cerebellar degeneration with anti-Yo antibodies and an associated submandibular gland tumor: a case report.

BACKGROUND: As a debilitating syndrome, paraneoplastic cerebellar degeneration (PCD) remains challenging to treat. Further, anti-Yo antibody (directed against human cerebellar degeneration-related protein 2) detection in patients with PCD is associated with unsatisfactory responses to existing therapies. Here, we present the case of a 60-year-old woman who developed PCD with anti-Yo antibodies and a submandibular gland tumor. CASE PRESENTATION: A 60-year-old woman presented with a 5-day history of unsteadiness of gait and inadequate coordination of her extremities, along with truncal instability. Although walking without aid was possible, dysmetria of all four limbs, trunk, and gait ataxia was observed. While routine biochemical and hematological examinations were normal, the patient's blood was positive for anti-Yo antibodies. When the neurological symptoms deteriorated despite administration of intravenous methylprednisolone, fluorodeoxyglucose-positron emission tomography (FDG-PET) and computed tomography (CT) images with contrast enhancement were performed, which showed a tumor in the left submaxillary gland. She underwent total left submandibular gland resection, including the tumor; histological and immunohistochemical results revealed a salivary duct carcinoma. She was administered intravenous methylprednisolone, followed by 10 plasma exchange sessions, intravenous immunoglobulins, and cyclophosphamide therapy. Following treatment, her symptoms were not alleviated, even after the reduction of anti-Yo titers. CONCLUSIONS: Although tumor detection was delayed, early tumor detection, diagnosis, and PCD treatment are essential because any delay can result in the progression of the disorder and irreversible neurological damage. Therefore, we recommend that the possibility of a salivary gland tumor should be considered, and whole-body dual-modality CT, including the head and neck, and FDG-PET should be performed at the earliest for patients with well-characterized paraneoplastic antibodies when conventional imaging fails to identify a tumor.

[A case of paraneoplastic limbic encephalitis associated with neck neuroendocrine carcinoma].

A 69-year-old man presented with a history of personality change for several years. He was admitted to our hospital due to partial seizure. A cerebrospinal fluid test and an electroencephalogram showed no specific abnormalities, but brain magnetic resonance imaging revealed abnormal findings in the right temporal pole, bilateral amygdala to hippocampus, and insular cortex. He was diagnosed with limbic encephalitis accompanied by partial seizure, and received infusion of an antiepileptic agent and acyclovir. Additional examinations for malignancy and autoimmune disease were performed, and neck CT and MRI revealed a neck tumor. Neck lymph node biopsy suggested lymph node metastasis of a neuroendocrine neoplasm derived from other organs. He did not want aggressive treatment involving surgical resection and chemotherapy, and thus, conservative treatment was chosen by an otorhinolaryngologist and immunotherapy was not used. After discharge, the neck tumor grew gradually. To manage the focal mass effect, chemotherapy and surgical resection followed by chemoradiotherapy were performed by the otorhinolaryngologist on days 244 and 325 of the disease course, respectively. Histology of resected tissues disclosed neck neuroendocrine carcinoma derived from a submandibular gland. His personality change improved temporarily after surgical resection, but then worsened again with regrowth of the tumor. He died on day 723. After death, a blood test revealed the presence of anti-amphiphysin antibody. This case suggests that neck neuroendocrine carcinoma can induce paraneoplastic limbic encephalitis, and in such cases, early surgical resection of the neck tumor with suspected lymph node metastasis is necessary both to control symptoms associated with encephalitis and to exclude carcinoma derived from the neck itself.

A gland of diverse pathology and unpredictable behaviour: our experience of primary submandibular gland malignancies.

Submandibular gland tumours are relatively uncommon tumours and demonstrate diverse histological types and a variable prognosis. The aim of this study was to analyze our experience with submandibular malignancies over a period of 6 years (January 2009 to December 2015). Patient data from the 6-year period were reviewed retrospectively and 51 patients with submandibular malignancies were identified. Demographic data, clinicopathological details, treatment received, complications, and follow-up were recorded. The mean age of the 51 patients at presentation was 49.1 years. They were followed up for a mean 20.3 months. Nine of 47 patients (19.1%) developed distant metastasis during follow-up, while only three (6.4%) developed local recurrence. Disease-free survival at 2 years was 69.7% and overall survival at the end of 2 years was 77.8%. Actuarial 5-year survival was 57.8% when all subtypes were considered. The overall mean time to recurrence was 10 months (6-24 months). Nodal positivity was the only prognostic factor that was significant on multivariate analysis, while age, sex, perineural invasion, and grade were not. With advances in surgical and radiotherapy techniques, loco-regional control rates have improved greatly; however, effective adjuvant treatment to prevent systemic relapse is still lacking.

Is it just a psoriasiform dermatitis?

Bazex syndrome (BS) is a rare paraneoplastic syndrome most frequently associated with squamous cell carcinomas of the upper aerodigestive tractand other tumours. Characteristically, cutaneous lesions precede the diagnosis of malignancy. We report a 72-year-old patient with 1-year history of acral dermatitis. The diagnosis of BS was based on the presence of psoriasiform acral dermatitis and the evidence of two simultaneous tumors (prostate adenocarcinoma and undifferentiated carcinoma ofthe submandibular gland). It is important to have this syndrome in mind since cutaneous features usually precede an underlying neoplasm.

Transcervical extirpation of the submandibular gland: the University of Marburg experience.

Surgical excision of the submandibular gland is the treatment of choice for lesions affecting this gland. The data of 87 patients, who underwent a transcervical extirpation of the submandibular gland as a single operation over the past 10 years at a single institution in Germany, were available for analysis. Sialolithiasis (73.5%) was the most common reason leading to excision, followed by benign (18.5%) and malignant tumors (8%). Complications included temporary palsies of the marginal mandibular branch of the facial nerve (5.7%), the lingual nerve (5.7%), and the hypoglossal nerve (1.1%), and wound infections in the form of hematoma (3.4%) and seroma (1.1%).

Management of sarcomatoid salivary duct carcinoma of the submandibular gland duct with coexisting seropositive human immunodeficiency virus.

BACKGROUND: Sarcomatoid salivary duct carcinoma of the submandibular gland is extremely rare. This paper highlights the impact of surgery and adjuvant radiation therapy on the outcome of this disease. METHODS: A 59-year-old man with human immunodeficiency virus presented with a painless, rapidly growing left neck mass. Biopsy followed by surgical excision of the left submandibular gland revealed sarcomatoid salivary duct carcinoma of the submandibular gland duct with perineural invasion and close margins, for which he underwent adjuvant radiotherapy. Post-operative positron emission tomography and computed tomography revealed no residual or metastatic disease. Pathological analysis of tumour-node-metastasis staging revealed a T2 N0 M0 (stage II) tumour. RESULTS: The patient tolerated his treatment without serious acute or long-term side effects. There was no evidence of disease on comprehensive examination or on positron emission tomography or computed tomography scans at the 4.6-year follow up. CONCLUSION: Surgery followed by adjuvant radiotherapy provided practical locoregional control with acceptable toxicity. Further detailed case reports are warranted to optimise the management of this rare malignancy.

First bite syndrome caused by adenoid cystic carcinoma of the submandibular gland.

First bite syndrome is a well-described sequelae of parapharyngeal space surgery, thought to result from sympathetic denervation of the parotid gland. We describe a case of first bite syndrome caused by an adenoid cystic carcinoma of the submandibular gland. The tumor was not clinically or radiographically apparent until 18 months after initial presentation despite repeated imaging. In patients with first bite syndrome and no surgical history, there must be high suspicion for a malignancy, which may be occult on presentation. The submandibular gland should be considered as a possible site of a lesion.

[A case of bilateral aplasia of the submandibular glands associated with a unilateral submandibular hemangioma].

Congenital aplasia of the major salivary gland is a rare condition. We report on a case of bilateral aplasia of the submandibular glands associated with a left submandibular hemangioma. A 62-year-old woman came to our department complaining of a 3-year history of left submandibular swelling. She had no notable family or personal medical history. On physical examination of the head and neck region, a 30 mm, non-tender mass was palpated in the left submandibular area. On imaging examinations including ultrasonography and CT, lack of the right submandibular gland and a left submandibular mass with calcification were demonstrated. Fine needle aspiration cytology resulted in blood elements only. Functioning tissue could not be observed in the bilateral submandibular glands on technetium pertechnetate scintigraphy. We performed a left submandibular tumor extirpation. Intraoperatively, the left submandibular gland and duct were missing. On pathologic examination, the tumor was found to be a hemangioma. The parotid and sublingual glands were recognized bilaterally on postoperative MRI. Her postoperative condition was satisfactory. This condition may be due to the dysfunction of several factors, such as fibroblast growth factors, related to gland differentiation.

Ipsilateral removal of sublingual gland after excision of submandibular gland for benign disease: 10-year prospective study and comprehensive review 1978-2008.

The removal of the submandibular salivary gland for non-neoplastic disease is a common procedure that has well documented risks and postoperative complications. Persistent symptoms of pain and swelling in the floor of the mouth that can occur after excision of the submandibular gland may require removal of the sublingual gland, but a causative association between the two has not, to our knowledge, been comprehensively established. We prospectively studied 77 patients who had had excision of the submandibular gland for benign disease, six of whom (8%) returned to theatre for ipsilateral sublingual sialadenectomy within a 5-year period after the initial operation (mean 24 months). These findings suggest that the association is under-reported, and may need to be considered during the consent process for excision of the submandibular salivary gland.

[Unusual pathology of submandibular gland--Küttner tumor]. / Rzadka patologia slinianki podzuchwowej--guz Küttnera.

Küttner tumor (chronic sclerosing sialadenitis) is a chronic, benign lesion of salivary gland. Its clinical course resamble malignant process. Authors report two typical cases of CSS affecting submandibular gland. We inform, that we start follow up of those cases consist of--us imaging /twice a year/, indication of LE and IgG level, protein electrophoresis.

Coexistence of salivary gland cysticercosis with squamous cell carcinoma of the mandible.

Cysticercosis is a parasitic infestation caused by the pork tapeworm larval stage, Cysticercus cellulosae. The majority of the cases present in ocular, cerebral, and subcutaneous locations. We report the presence of cysticercosis inside the submandibular gland in association with squamous cell carcinoma of the inferior alveolar ramus of the mandible. To the best of our knowledge, this is the first case report documenting cysticercosis inside a salivary gland. A 65-year-old male presented with complaints of an ulcerative lesion on the inferior alveolar ramus present for 2 months. Histological examination revealed a keratinizing well-differentiated squamous cell carcinoma involving the alveolar margin and mandible. The histopathological examination of the submandibular gland revealed cysticercosis. This case emphasizes the importance of adequate sampling of all the tissues obtained for associated infectious disorders, more so in immunosuppressed patients, which will help the clinician to manage the case appropriately.

A case report of coexistence of a sialolith and an adenoid cystic carcinoma in the submandibular gland.

The occurrence of sialoliths in the submandibular gland is 80% due to the specific anatomy of both the gland and its duct. The diagnosis is rather easy because of the obvious clinical signs of the entity. Imaging studies are always necessary in order to treat the patient as effectively as possible. The stones do not tend to occur within the gland as frequently as in the respective duct. The coexistence of sialoliths and malignant tumors is extremely rare. A 70-year-old woman with intraparenchymal stone was operated in our ENT department. In addition to the sialolith the pathological examination revealed the existence of an adenoid cystic carcinoma (ACC), that extended to the neighboring skeletal muscle. This is the reason why we believe it would be useful to report this case of a large stone (14 mm in diameter) located in the submandibular gland coexisting with ACC. This case report is a very good example illustrating that all available means should be used prior to reaching a conclusion and making a health professional decision.

[Sjogren syndrome: a review of the literature and a case report]. / Le syndrome de Sjögren: revue de littérature et case-report.

Sjögren syndrome is one of the most prevalent autoimmune diseases in which the body's immune system mistakenly attacks its own moisture producing glands. Although Sjögren syndrome occurs in all age groups in both women and men, women in their fourties are the most affected. Sjögren's syndrome can occur alone or in the presence of another connective tissue disease, respectively called primary and secundary Sjögren syndrome. When two of the three clinical hallmarks: keratoconjunctivitis sicca, xerostomia or connective tissue disease are present, Sjögren 's syndrome should be considered. To confirm the diagnosis of Sjögren's syndrome several tests are required. e.g. blood tests, ophthalmologic tests and oral tests. Rheumatologists have the primary responsibility for managing Sjögren's syndrome. Other specialists can treat the related symptoms. The incidence of lymphoma is higher in patients with Sjögren's syndrome than in the general population. Therefore patients must be monitored carefully for the development of related autoimmune diseases, lymphoma and other complications. Sjögren's syndrome is serious but generally not fatal if complications are diagnosed and treated early.

[A case of bilateral oncocytomas of the submandibular gland and synchronous renal oncocytomas]. / Przypadek równoczesnego wystepowania guzów onkocytarnych slinianek podzuchwowych z onkocytoza nerek.

A case of 42 years-old man with bilateral big sized (12 x 5 cm and 10 x 4,5 cm) oncocytoma in submandibular glands was presented. Oncocytoma is a rare benign neoplasm observed in numerous of organs. In the case described besides submandibular glands tumours, accessory oncocytic tumours in both kidneys were diagnosed. It was the reason of earlier unilateral nephrectomy. Surgical treatment was applied. The comparison of the both tumours histology allowed to establish proper diagnosis. The case is presented because of the explicitly of the oncocytoma location especially in the both submandibular glands simultaneously with tumour of the same histology in distant organs.

Marginal zone B-cell lymphoma of the salivary gland arising in chronic sclerosing sialadenitis (Küttner tumor).

We report a case of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type of the salivary gland arising in a background of chronic sclerosing sialadenitis. Chronic sclerosing sialadenitis is a common fibrosing chronic inflammatory lesion of the submandibular gland, which is thought to be the result of sialolithiasis, and is not associated with a systemic autoimmune disease. Salivary MALT lymphomas are typically associated with lymphoepithelial sialadenitis (LESA) in a patient with or without Sjögren's syndrome. Our case of salivary MALT lymphoma was neither preceded by Sjögren's syndrome nor accompanied by LESA. This case suggests that chronic inflammatory processes other than Sjögren's syndrome may provide a substrate for the development of MALT lymphoma.

Intraoral removal of the submandibular gland: a new surgical approach.

The transcervical procedure used for treating sialolithiasis in the submandibular gland has been generally accepted by head and neck surgeons. However, several clinical problems after surgery through the transcervical approach have been described. We introduce a new surgical approach for excision of the submandibular gland indicated in the chronically inflamed salivary gland with or without calculus and benign mixed tumor of the submandibular gland. Thirty-one cases of submandibular gland excision through the intraoral approach were reviewed, analyzing surgical technique and morbidity. Early postoperative discomforts developed, such as a temporary lack of function of lingual nerve and a temporary limitation of tongue movement, but recovery was within a short period of time in all patients involved. No symptomatic late complications appeared, such as residual inflammation of Wharton's duct and neurologic sequelae. We suggest that this approach can be extended to the excision of the submandibular gland as an alternative to the transcervical approach. The major advantages of this approach are the avoidance of an external scar and injury to the marginal mandibular nerve or the hypoglossal nerve.