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.

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%).

[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.

[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.

[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.

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.

[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.

Malignant epithelial tumours associated with autoimmune sialadenitis.

Malignant epithelial tumours associated with autoimmune sialadenitis are rare in white races but occur more often in those of Eskimo or oriental descent. Ultrastructurally these tumours are squamous in origin, and they may arise from the epithelial component of autoimmune sialadenitis. The two cases reported are the first described in natives of this country, and in one, a case of parotid tumour, autoimmune sialadenitis preceded the development of undifferentiated carcinoma by 12 years; the other, a submandibular lesion, indicates some diagnostic difficulties that were found. This condition deserves wider recognition, as adequate primary treatment may result in long term survival.

Adenoid cystic carcinoma of the submaxillary gland.

A review of submaxillary gland neoplasms over a 21 year period revealed 15 malignancies and 7 benign tumors. All of the malignancies occurred in females, and 11 of these were adenoid cystic carcinoma. Two of the 11 were found to have infiltrating ductal carcinoma of the breast (1 pre and 1 postdiagnosis of the submaxillary carcinoma) and 3 had benign breast disease. While previous reports have suggested an association of parotid gland neoplasia and breast cancer, this is the first known report of an association between adenoid cystic carcinoma of the submaxillary gland and cancer of the breast. The discussion of adenoid cystic carcinoma of the submaxillary gland emphasizes the increased frequency of this disease in females, its association with breast disease, and also experimental submaxillary gland neoplasia.

Hemiageusia, hemianaesthesia and hemiatrophy of the tongue.

A patient with a submandibular gland carcinoma was found clinically to have a unilateral chorda tympani, lingual and hypoglossal nerve deficit. This unique neurological entity of loss of taste sensation of one-half of the tongue (hemiageusia), hemianaesthesia and hemiatrophy of the tongue, has not previously been reported.

[Stent implantation as a palliative therapeutic measure in stenosing tumors of central airways]. / Stentimplantation als palliative Therapiemassnahme bei stenosierenden Tumoren der zentralen Atemwege.

PURPOSE: Stent implantation was used to treat patient with malignant tracheobronchial obstructions to determine the effectiveness in producing symptomatic palliation. METHODS: 18 patients (15 men and three women; median age 57 years) with malignant tracheobronchial stenosis were treated by application of metal stents (15 Palmaz-, 10 Gianturco-, 4 Wallstents). The indication for stent implantation was given in 13 patients by clinically significant dyspnea, besides in 5 patients by therapy resistant postobstructive pneumonia. RESULTS: In 17 patients correct positioning of the stents was achieved and the symptoms completely disappeared until tumor related death. Median survival was 137 days (min. 10 days to max. 322 days). In one patient symptoms recurred three months after stent implantation. CONCLUSION: The application of metal stents in patient with malignant tracheobronchial obstruction appears to be a useful palliation procedure. The treatment was well tolerated and very effective.

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.

Choristoma of the submandibular gland: a rare cause of cervical swelling.

A case of choristoma of the submandibular gland excised from a 4-week-old female infant is reported. The differential diagnosis is discussed and suggestions are made for the possible etiology of the lesion.

Cavernous haemangioma of the submandibular salivary gland.

Salivary gland haemangioma is more common in the parotid than in any other salivary gland. Parotid haemangioma is considered to be the most common salivary gland tumour in infants, but is also occasionally encountered in adults. A case of cavernous haemangioma affecting the submandibular salivary gland in an adult woman is presented. The striking histological feature is the presence of numerous vascular channels assuming a periductal arrangement.

Synchronous pleomorphic adenomas of the major salivary glands: a case report.

The presentation of multiple distinct tumors in major salivary glands is rare. Although the most common tumor with bilateral synchronous or metachronous development is the Warthin tumor, pleomorphic adenomas have been diagnosed simultaneously as well. We report the case of a female patient who was diagnosed with pleomorphic adenomas in the right parotid and submandibular glands, concomitant with sialolithiasis affecting the submandibular gland. This patient had been exposed to head and neck radiotherapy in childhood, which may have played a role in the development of her tumors. A review of the relevant literature is included.

Facial sensory loss--a need for suspicion.

Three patients with facial sensory loss were referred by their general dental practitioners. In the absence of local or systemic factors to account for such symptoms, the dental surgeon should be alert to the possibility of sinister underlying pathology, possibly remote from the jaws. Even in the face of negative special tests, patients should be thoroughly investigated and referred accordingly. Following referral, an exhaustive search should be undertaken for possible causes, particularly for the possibility of metastatic disease.

[A case of adenoid cystic carcinoma manifesting Garcin's syndrome--effectiveness of cerebrospinal fluid ferritin as a tumor marker in malignant CNS involvement].

We reported a 69-year-old man exhibiting Garcin's syndrome caused by adenoid cystic carcinoma of the right submandibular gland. The patient first experienced abnormal sensations in his right cheek, and the cranial nerves on his right side gradually became affected. He was admitted for hoarseness and dysphagia. Physical examination revealed a right submandibular mass, and neurological examination revealed that the first cranial nerve and the fifth to twelfth cranial nerves on the right side were affected. Laboratory examination showed a rise of both serum and cerebrospinal fluid (CSF) ferritin, suggesting an intracranial invasion of the submandibular tumor. But other tumor markers, CSF protein and cell counts, CSF pathologic study and radiological studies for the central nervous system (CNS) were all negative. A submandibular tumor biopsy revealed adenoid cystic carcinoma. The radiation therapy, including the skull base, provided relief of the patient's symptoms, the tumor was reduced and serum and CSF ferritin level decreased. It is possible that CSF ferritin is a sensitive marker for CNS involvement of malignant tumor because of its permeability of the blood brain barrier and the absence of correlation between serum and CSF.