Mycosis fungoides refractory to treatment - importance of a multidisciplinary approach.

We report a case of difficult-to-control mycosis fungoides (MF), where the role of the dental surgeon was crucial for the control and prognosis of the disease. A 62-year-old female patient diagnosed with MF had a previous record of red patches and small raised bumps on the face, along with a cancerous growth in the cervical and vulvar region. The patient was initially treated with methotrexate and local radiotherapy without resolution. Chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone was then started (CHOP protocol). The dental team of a reference hospital was consulted to evaluate swelling in the anterior region of the palate, which had been developing for two months, reporting discomfort when eating. The role of the dentistry team was fundamental in the differential diagnosis of oral lesions with dental infections, second neoplasia, or even a new site of disease manifestation, in addition to controlling mucosal changes resulting from chemotherapy. After ruling out dental infection, the dentistry team performed a lesion biopsy to confirm the diagnosis. The histopathological and immunohistochemical analysis showed atypical lymphoid infiltration of T cells (CD3+/CD4+/CD7-/CD8-), coexpression of CD25, and presence of CD30 cells, corresponding to the finding for MF. Identifying CD30 + allowed for a new chemotherapy protocol with brentuximab vedotin (BV) combined with gemcitabine. This protocol effectively controlled MF, which previous protocols had failed to do. The diagnosis by the dental team was essential for therapeutic change and improvement of the patient's clinical condition without the need for invasive medical procedures.

Striking enhancement at the site of radiation for nivolumab-induced Stevens-Johnson syndrome.

Stevens-Johnson syndrome is a rare adverse cutaneous drug reaction characterized by epidermal detachment of <10% body surface area with an average mortality rate of 1-5%. The mechanism of SJS is not fully understood. Nivolumab is a monoclonal antibody directed against programmed cell death-1 protein (PD-1), a receptor with immune checkpoint inhibitory and antineoplastic activities. We present a case of SJS in a patient being treated with anti-PD-1 therapy nivolumab for metastatic squamous cell carcinoma of the oropharynx. This case is unusual because of the severe accentuation with striking enhancement at his prior radiation site and in the cutaneous region with heavier tumor burden from his metastatic disease. This reaction may give insight to the underlying pathophysiology of SJS, suggesting that immune checkpoint inhibitors can activate T-cells to target keratinocytes and that external factors may be involved in creating distinct epitopes for T-cell recognition. We hope this case adds to the body of knowledge in the pathogenesis of Stevens-Johnson syndrome and cutaneous adverse events seen with checkpoint inhibitors.

Kindler syndrome complicated by invasive squamous cell carcinoma of the palate.

INTRODUCTION: Kindler syndrome is a very rare, autosomal recessive genodermatosis characterized by skin fragility and photosensitivity in infancy with progressive poikiloderma. CASE REPORT: We report the case of a young woman with a history of Kindler syndrome predominantly characterized by extensive involvement of the oropharyngeal mucosa. The patient presented with an ulcerative lesion of the palate. Computed tomography and biopsy concluded on unresectable invasive squamous cell carcinoma of the hard palate. Neoadjuvant chemotherapy was proposed, but the patient died after the first course of chemotherapy in a context of severe gastrointestinal mucositis and generalized sepsis. DISCUSSION: Mucosal manifestations of Kindler syndrome have been described in the literature, but very few cases of malignant transformation to squamous cell carcinoma have been reported, although it is a very well known, long-term complication of this disease. To our knowledge, this is the second reported case of Kindler syndrome complicated by invasive squamous cell carcinoma of the hard palate.

Propranolol and prednisolone combination for the treatment of segmental haemangioma in PHACES syndrome.

Posterior fossa malformations-haemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe syndrome (also known as PHACES syndrome) is a rare neurocutaneous disorder. Children presenting with these manifestations need careful ophthalmological, cardiac and neurological assessment. They may have one or more of these extracutaneous manifestations, the most common being cerebral and cardiovascular anomalies. There is controversy about treating these children with propranolol especially if they have cerebrovascular involvement with narrow, dysplastic or absent blood vessels. The concern with propranolol is that hypotension may lead to reduced cerebral blood flow and neurological consequences. Prior to propranolol the systemic treatment for haemangiomas was prednisolone and then the concern was the opposite, namely hypertension. Our proposal was whether a combination of these two drugs would provide a safer and faster recovery. We report three retrospective cases of PHACES syndrome, each of whom received treatment with a combination of propranolol and prednisolone: two children were started on prednisolone and propranolol was added because the haemangiomas failed to respond adequately; the third child was started on propranolol and developed peripheral ischaemia and ulceration necessitating a reduction in dose addition of a low dose of prednisolone. All three patients, who failed on the one treatment, responded well to combination therapy without any significant complications. These outcomes suggest that for some patients with PHACES syndrome the use of combination treatment with propranolol and prednisolone could be advantageous, potentially allowing for the introduction of low doses of each with an enhanced combined effect. The doses can be increased gradually depending on the magnetic resonance imaging findings.

Paranasal sinus metastasis of breast cancer.

A 76-year-old woman presented with symptoms suggestive of acute sinusitis. Previously, her breast carcinoma was treated with right lumpectomy, adjuvant chemotherapy and breast radiotherapy. She remained free from recurrence for the following 8 years. After initial treatment with antibiotics, the local symptom worsened with exophthalmos, eye blindness and development of an ulceration of the hard palate. MRI showed irregular enhancement of the nasal cavity extended to the maxillary sinus and ethmoidal lamina and concomitant infiltration of the orbit and skull base. A biopsy of the palatal ulcer showed a poorly differentiated adenocarcinoma and was compared with the histology of the primary breast tumour and it was concluded for the same morphology. After discussion at the multidisciplinary team, a specific chemotherapy has been activated with an initial local response. Further surgical resection was not thought appropriate and the patient has subsequently undergone palliative radiotherapy to the right paranasal lesions to improve local disease control.

Diffuse swelling of the buccal mucosa and palate as first and only manifestation of an extranodal non-Hodgkin 'double-hit' lymphoma: report of a case.

BACKGROUND: Most of the lymphomas arising in the oral cavity are of B-cell origin. Among these, diffuse large B-cell lymphomas are the most common. Diffuse large B-cell lymphomas may exhibit more than one chromosomal rearrangement and are then referred to as 'double-hit' or 'triple-hit' lymphomas. CASE REPORT: We present a case of an intraoral 'double-hit' lymphoma in a 76-year-old male who had been referred by an oral surgeon in private practice. Intraoral examination exhibited a firm, exophytic lesion in the region of the right hard palate and buccal mucosa with extension to the soft palate. Radiographic examination exhibited a massive thickening of the right sinus membrane, and arrosion of the lateral and basal cortical sinus walls in the right maxilla. After diagnosis of the lesion, the patient was treated with six cycles of chemotherapy. DISCUSSION: Lymphomas arising within the oral cavity account for less than 5% of all oral malignancies and typically affect the palatine tonsils and the palate. 'Double-hit' lymphomas are associated with older age, usually present with an advanced stage of disease, and show an aggressive clinical behaviour. They normally have a poor prognosis, even when treated with intensive chemotherapy regimens. Nevertheless, in the case presented, the patient was free of symptoms 1 year after initial diagnosis.

Intensity-modulated radiotherapy in the treatment of oropharyngeal cancer: an update of the Memorial Sloan-Kettering Cancer Center experience.

PURPOSE: To update the Memorial Sloan-Kettering Cancer Center's experience with intensity-modulated radiotherapy (IMRT) in the treatment of oropharyngeal cancer (OPC). METHODS AND MATERIALS: Between September 1998 and April 2009, 442 patients with histologically confirmed OPC underwent IMRT at our center. There were 379 men and 63 women with a median age of 57 years (range, 27-91). The disease was Stage I in 2%, Stage II in 4%, Stage III in 21%, and Stage IV in 73% of patients. The primary tumor subsite was tonsil in 50%, base of tongue in 46%, pharyngeal wall in 3%, and soft palate in 2%. The median prescription dose to the planning target volume of the gross tumor was 70 Gy for definitive (n = 412) cases and 66 Gy for postoperative cases (n = 30). A total 404 patients (91%) received chemotherapy, including 389 (88%) who received concurrent chemotherapy, the majority of which was platinum-based. RESULTS: Median follow-up among surviving patients was 36.8 months (range, 3-135). The 3-year cumulative incidence of local failure, regional failure, and distant metastasis was 5.4%, 5.6%, and 12.5%, respectively. The 3-year OS rate was 84.9%. The incidence of late dysphagia and late xerostomia ≥Grade 2 was 11% and 29%, respectively. CONCLUSIONS: Our results confirm the feasibility of IMRT in achieving excellent locoregional control and low rates of xerostomia. According to our knowledge, this study is the largest report of patients treated with IMRT for OPC.

Approach to benign tumors of the palate: analysis of 28 cases.

We conducted a retrospective analysis of 28 patients-15 men and 13 women, aged 17 to 71 years (mean: 41.6)-who had undergone surgery for the treatment of a benign tumor of the hard or soft palate. The most common chief complaint was a palatal mass, which was reported by 14 patients (50.0%). Tumors were more common in the hard palate than in the soft palate by a margin of 23 to 5 (82.1 to 17.9%; p = 0.001). The most common histopathologic diagnosis was pleomorphic adenoma, which occurred in 9 cases (32.1%). Most patients were treated with local excision with clear margins, which was sufficient in almost all cases, as there were only 2 recurrences, both of which occurred in men with a hard-palate pleomorphic adenoma. For these 2 patients, a wider excision and repair with palatal islet flaps was performed, and no further recurrence or malignant transformation was observed during follow-up. Two patients with a soft-palate hemangioma were treated with an intralesional steroid injection and radiofrequency ablation, which reduced the size of their lesion considerably.

Granulocytic sarcoma of palate. Case report and review of literature.

A rare case of granulocytic sarcoma of the hand and palate, also known as chloroma, occurring in an adolescent patient is presented. Diagnostic clinical criteria, along with treatment pictures, are also reviewed.

Synchronous squamous cell carcinoma and chronic lymphocytic leukemia of the palate.

Although multiple synchronous malignancies of the same histological type have been described frequently in the upper aerodigestive tract, this is a less frequent phenomenon when dealing with tumors of different lineage. We present a case of a man who developed simultaneously an oral squamous cell carcinoma and chronic lymphocytic lymphoma of the palate without any previous risk factors. To the best of our knowledge, this is the first case well documented in the literature of synchronous oral squamous cell carcinoma and chronic lymphocytic lymphoma of the palate in the same sample. The presence of multiple primary malignancies of different histological types not only complicates the treatment but also worsens the prognosis of the patient.

Carcinoma ex pleomorphic adenoma of soft palate with cavernous sinus invasion.

BACKGROUND: Carcinoma ex pleomorphic adenoma (CXPA) is an aggressive salivary gland malignancy and rare in minor salivary gland. A soft palate CXPA initially presenting as direct cavernous sinus (CS) invasion is very rare. CASE PRESENTATION: A 60-year-old male had a 3-month history of a small soft palatal mass with progressing left cheek numbness, proptosis, and disturbed vision. Biopsy of soft palatal tumor showed pleomorphic adenoma. Magnetic resonance imaging showed a tumor involving left maxilla, and extended from pterygopalatine fossa, inferior orbital fissure to CS. Excision of tumor revealed CXPA. Adjuvant concomitant chemo-radiation therapy (CCRT) was given. The tumor recurred 5 months later in left CS which was re-treated with CCRT. The disease status was stable at 2 years after the diagnosis of CXPA. CONCLUSION: We present this case to emphasize that patients with symptoms such as facial numbness, proptosis and disturbed vision should be carefully investigated for lesions invading CS by perineural spread.

The use of pedicled temporal musculoperiosteal flap with or without free calvarial bone graft in maxillary reconstructions.

Various techniques have been used to repair maxillary defects. The aim of this study was to evaluate the suitability of pedicled temporal musculoperiosteal flap (PTMF) and free calvarial bone graft for the reconstruction of maxillary defects. In this retrospective series, 34 patients operated on from 1995 to 2006 at Turku University Central Hospital because of defects of maxilla reconstructed using PTMF with or without free calvarial bone graft were evaluated. The diagnosis, the indication for surgery, the location and staging of the tumours, and the type of radiotherapy used were reviewed. The classification of the maxillary defects was performed according to the classification of Brown (Br J Oral Maxillofac Surg 40:183-190, 2002) and the success rates of the reconstructions were evaluated. Of the patients, 32 had been operated on due to a malignant tumour, one due to a benign tumour and one due to posttraumatic palatal defect. Preoperative radiotherapy (n = 14), preoperative chemoradiotherapy (n = 2) or postoperative radiotherapy (n = 11) had been used in the tumour group. As a reconstructive method, PTMF had been used with (n = 21) or without (n = 13) free calvarial bone graft. The use of free calvarial bone graft did not have a significant effect on flap survival. At 1-month follow-up, the flap survival in the 32 patients was 71.9%, whereas 28.1% of the patients suffered from partial flap loss, but there was no total flap loss. At 6-month follow-up, the flap survival in 26 patients was 76.9%, whereas 7.7% of the patients suffered from partial flap loss, and there were four (15.4%) total flap losses. If unilateral alveolar maxillectomy had been performed (Brown classification a), at 1-month follow-up, the flap survival was 82.6%, 17.4% of the patients suffered from partial flap loss, and there was no total flap loss. At 6-month follow-up, the flap survival was 89.5%, while 10.5% of the patients suffered from partial flap loss, and there was no total flap loss. The application of PTMF with or without free calvarial bone graft for reconstruction of limited palatal and maxillary defects appears to be feasible.

Palatal swelling as the first and only manifestation of extranodal follicular non-Hodgkin lymphoma: a case presentation.

Non-Hodgkin lymphomas (NHLs) in the head and neck region are malignant lymphoid neoplasms that usually originate from B-lymphocytic cell lines. Primary extranodal manifestations of this hematolymphoid tumor in the oral cavity are rare and involve the maxillary jaw including the palatal soft tissues, the mandible, and gingival tissues in patients between 60 and 70 years of age without sex predilection. This case report of an extra-nodal NHL in the palate of a 75-year-old patient emphasizes the importance of accurate clinical, radiographic, and histologic diagnostic procedures to avoid delayed diagnosis or inappropriate treatment strategies. Chemotherapy, radiotherapy, or a combination of the two with a regular clinical and hemic follow-up is recommended.

Acute hearing loss, dysarthria, dysphagia, and a rubbery intraoral mass in an 18-year-old woman.

Rhabdomyosarcoma is the most common soft tissue tumor of childhood, frequently presenting in the head and neck, genitourinary tract, or extremities. We present a case of rhabdomyosarcoma in which an 18-year-old woman presented with abrupt onset unilateral hearing loss, tinnitus, dysarthria, dysphagia, and a new painless red bump on the palate. With an alveolar subtype and older age, both predictors of poor prognosis, early recognition of disease of these symptoms is vital.

Disseminated Kaposi's sarcoma and HCV infection: only a casual relationship? A case report.

The authors present a case of disseminated Kaposi's sarcoma in a male patient, HIV negative and Hepatitis C virus (HCV) positive. Although it is well-known that in HCV positive patients the onset of cutaneous diseases such as porphyria cutanea tarda, mixed essential cryoglobulinemia, lichen planus, polyarteritis nodosa, itch/prurigo, is possible, papers on its association with disseminated Kaposi's sarcoma in HIV negative patients are rare in the literature. Such an association is probably not a matter of chance: in fact, the changes to the immune system induced by the HCV virus, in synergy with those induced by the Human Herpetic virus-8, could likewise play a role in the development of Kaposi's sarcoma as happens in patients with immunodeficiency .