Merkel cell carcinoma of the forehead area: a literature review and case report.

BACKGROUND: Merkel cell carcinoma (MCC) is an uncommon, aggressive malignancy of the skin, mostly affecting head and neck area in elderly white patients. Between head/neck sites, face accounts for 61% and forehead accounts for 17% of all face MCCs. PURPOSE: We here present a literature review MCC cases arising in the forehead area, published in the English literature in the period 1987-2018, and report a personal observation with a late diagnosis and a treatment out of the current recommendations. The aims of this paper are to provide an up-to-date on MCC arising in the forehead area and to raise awareness about misdiagnosis of this type of lesion mimicking arteriovenous malformations (AVM). MATERIAL AND METHOD: Literature review was performed on PubMed and Medline database and "Merkel cell carcinoma (MCC)," "forehead" and "MCC forehead location" were the terms the authors searched for. Patients' data have been drawn from descriptions of single cases and of short case series reports. For each case, data were collected about clinical characteristics, treatment modalities and outcomes. The study has been limited to the clinical features of the disease, excluding etiologic/pathogenic aspects. RESULTS: Twenty-five patients with forehead MCC have been identified, coming from 20 sources. Nineteen presented a locoregional disease and 6 had an advanced pathology. TNM classification was reported in only two cases lacking for the other available data. Patients presented at mean age of 66 years with solitary or multiple nodules or dome-shaped/hemispherical mass, rarely ulcerated. Mean size of tumors was 1.13 cm of max diameter. Previous or concurrent malignancies or immune-hematologic disorders (AIDS) were often associated. At first investigation, lesion was often mistaken for other malignant or benign processes and, then, diagnosis was generally late. Some type of preoperative biopsy was performed in 3 patients, while the others had only a postoperative microscopic study of specimen. Initial treatment consisted in 6 cases (24%) in a not further specified about extent and width of margins local excision of the primary lesion, while a wide resection was reported in only 3 cases (12%). Surgical treatment of involved lymph-nodes was performed in 3 cases (12%). Six patients underwent radiotherapy for locoregional or distant recurrences. Mortality and overall survival rate at five years were 28% and 24%, respectively. Spontaneous regression was observed in 3 patients (12%). CASE REPORT: Personal observation concerned an 82-year-old woman presenting with a forehead periorbital 5 × 5 cm red-bluish mass. The erythematous lesion was erroneously diagnosed as hemangioma on the base of color, the absence of any signs of malignancy, an angio CT indicating a hypervascular tissue and a FNA cytology (FNAC) lacking of malignant cells. The mass was excised as a benign lesion with about 1 cm margins extent without searching larger edges. Postoperative radiotherapy was offered to the patient after histology report, but she refused. After 4 months from surgery, she had a parotid metastasis and died from the illness in spite of platinum-based chemotherapy. CONCLUSIONS: This study confirms the aggressiveness of forehead MCC, comparable with that of other face similar tumors. Personal case suggests that the deceitful benign feature of lesion may mimic an AVM and that FNAC may be misleading and diagnostic failure worsen prognosis. Our experience suggests that in the face smaller than 2-3 cm margins resection may increase the risk of locoregional recurrence. Therefore, postoperative wide-field irradiation should be ever delivered, after forehead MCC surgery, not only when clear margins are unattainable or involved with tumor, but also when negative microscopic edges are documented and residual cancer is thought not persist in the tumor bed. Orbit irradiation seems to be not dangerous for the eye.

Superior Orbital Fissure Syndrome in Lateral Orbital Wall Fracture: Management and Classification Update.

The superior orbital fissure syndrome (SOFS) is an uncommon complication rarely occurring in association with craniofacial trauma. Work-up of a patient injured by a traumatic right orbitozygomatic complex fracture and SOFS is presented. Accurate computed tomography scan and three-dimensional reconstruction showed a medial displacement of the lateral orbital wall, compressing the right superior orbital fissure (SOF), without intraorbital bone fragment displacement or hemorrhage. Imaging also revealed a frontosphenotemporal fracture, according to Pellerin et al, that is, frequently associated with visual impairment. Our primary choice of therapy was a corticosteroid treatment in association with an early surgical approach. It consisted in en bloc reduction and osteosynthesis of the fracture through a bicoronal approach, recovering SOF size. A prompt and almost complete recovery of the abducens movement, without diplopia, was achieved in 1 week. The authors discuss indications and management of SOFS. The presence of fractures should urgently lead to surgery. We deny waiting for a medical treatment result, while preferring the prompt reduction of the fractures and extrication of the soft tissues. The main focus of this study is on patient's anatomical feature and fracture patterns.

Primary parapharyngeal tumours: a review of 21 cases.

BACKGROUND: The diagnostic and therapeutic procedures performed in a series of patients with primary parapharyngeal space (PPS) tumours treated at the ENT Departments of San Giovanni Bosco Hospital, Turin, and of the Pugliese-Ciaccio Hospital, Catanzaro, Italy, in the period 2001-2010 are evaluated. MATERIALS AND METHODS: The retrospective review included 20 patients, 11 male and 9 female, average age of 41 years operated on for 21 primary PPS tumours. The most common tumours found were neurogenic neoplasms, while those of salivary origin were the next most common. RESULTS: There were 14 paragangliomas (7 originating from carotid glomus, 5 from vagal and 2 from tympanicum), 1 sympathetic chain schwannoma and 6 pleomorphic adenomas. All the tumours were benign in nature and gave rise to few signs or symptoms. Patients underwent preoperative computed tomography (CT) scan or magnetic resonance imaging (MRI) or both. Most contrast-enhanced masses were submitted to some type of angiography. Most of the surgeries were planned through imaging alone, as preoperative fine needle aspiration (FNA) biopsy was performed only in six cases. Four different approaches were adopted for tumour removal: transcervical, transcervical/transparotid, cervical-transparotid-transmandibular and infratemporal fossa approach. There was no operative mortality, though neurologic morbidity was significant. Follow-up, extended to a maximum of 11 years, did not reveal any recurrences. In conclusion, neurogenic tumours may be the most common of PPS masses. Surgery is the mainstay treatment and external approaches offer the potential for satisfactory tumour resection. Of such external approaches, transcervical and cervical/transparotid are the most often used in benign forms. CONCLUSION: The number of perioperative complications encountered in this series confirms the difficulty of performing surgery in this complex area, even in benign cases. The chances of avoiding vascular damage and saving the trunks or most of the nerve fibres involved depend not only on the skill and experience of the surgeon but also on the anatomy of the lesion, the type of connection between the tumour and the nerve from which it originates and the distribution of neural fibres in or around the tumour mass.

Sentinel node radioguided biopsy in surgical management of the medullary thyroid carcinoma A case report.

INTRODUCTION: Medullary thyroid cancer is a rare carcinoma. Surgery is the only curative treatment and since cervical lymphnodes metastases are frequent and can occur at an early stage, a standardized central lymphnode dissection is associated to total thyroidectomy. However, the extent of lymphadenectomy to the lateral neck lynphnodes remains debated. To reduce the extent of lymphnode excision, the sentinel node biopsy has been used as an accurate technique to assess the status of the lymphnodes in the regional drainage basin in solid tumors, and more recently, in thyroid carcinoma. In this case report, we show the utility of the radioguided biopsy of the sentinel lymphnode in the surgical management of the medullary thyrod cancer. CASE REPORT: We present the case of a 24-year-old Caucasian, Italian woman with a sporadic medullary thyroid microcarcinoma occasionally detected by neck ultrasound and diagnosed by high serum calcitonin level and fine needle aspiration cytology. There was no ultrasound evidence of lymphnode involvement both in central and lateral compartment of the neck. We performed a preoperative mapping of the the sentinel lymphnodes by the injection of technetium-99m radiolabelled albumin nanocolloids in the thyroid nodule. Then our patient underwent total thyroidectomy combined with radioguided biopsy of the sentinel lymphnodes. Histology confirmed the presence of the medullary thyroid cancer and revealed micrometastases only in two sentinel lymphnodes detected in right lateral compartment of the neck so an ipsilateral lateral neck dissection besides the central neck dissection was performed at the end of operation. Basal and pentagastrin-stimulated serum calcitonin level was undetectable during the follow-up investigations. CONCLUSION: This is the first reported case that shows the utility of the radioguided SLN biopsy for the accurate staging of the cervical lymphnode involvement in patient with sporadic medullary thyroid microcarcinoma. Total thyrodectomy and central neck dissection is recommended for all patients with medullary thyroid carcinoma, but the indication for the lateral neck dissection is still controversial. The radioguided SLN biopsy technique could be a useful tool to perform the dissection only in those patients with proven lateral neck lymphnode involvement and reduce the extention of the lateral lymphnode excision and the incidence of related complications.

The platysma myocutaneous flap (PMF) for head and neck reconstruction: a retrospective and multicentric analysis of 91 T1-T2 patients.

The platysma myocutaneous flap (PMF) was first applied to intraoral reconstructions in 1978. PMF is not only an alternative to microvascular flaps but it also represents an excellent reconstructive choice especially in cases where free tissue transfer cannot be carried out. Failure and complications rate have been described as varying from 18 to 45% and this is why this flap should not be used in specific cases such as in the presence of cervical metastases and in cases of mandibulectomy and simultaneous reconstruction with alloplastic materials. The purpose of this study is to examine the experience and results obtained in three different and independent institutes where PMF has been adopted in 91 patients for head and neck cancer reconstructions. The authors report their departments' separate but simultaneous experiences with PMF for small and middle-size soft tissue defects in a 10-year period.

Development of a regional database for studying epidemiology of maxillofacial trauma.

This article discusses the development of the first regional computerized database for the epidemiological evaluation of maxillofacial trauma and tests its usefulness by evaluating the appropriateness and completeness of the resulting information.The database was developed using Microsoft Access, implemented using Visual Basic. Data were entered in the database by 4 different maxillofacial specialists, one from each of the 4 main regional hospitals where maxillofacial trauma is treated. Clinical information was taken from 100 complete records of patients hospitalized for maxillofacial fractures at the Maxillofacial Division of San Giovanni Battista Hospital in Turin from January to June 2009.Thirteen database fields were used: general information, cause and mechanism of injury, fracture site, Facial Injury Severity Scale, head and neck examination, associated injuries, timing and type of surgery, and days of hospitalization.Overall, the data entered were 99.45% complete and 99.5% accurate. Thus, our regional maxillofacial database can be considered complete and accurate. Some of the errors, mainly in the fields "fracture site" and "Facial Injury Severity Scale," were attributable to an incorrect interpretation of facial fracture diagnoses, based on the medical records that were provided.

Massive upper gastrointestinal bleeding from a pancreatic pseudocyst rupture: a case report.

INTRODUCTION: Bleeding from pancreatic pseudocyst's rupture into adjacent organs is a rare, but potentially fatal, complication of chronic pancreatitis requiring quick management. Timing of the rupture is unpredictable; early diagnosis and correct management is essential in preventing the bleeding. CASE PRESENTATION: We describe the case of a 53 years old male patient successfully treated with emergency surgery for massive hematemesis due to a rupture of a bleeding pseudocyst into the stomach. Patient underwent emergency laparotomy and suture of the bleeding vessel. At 5 years follow-up patient is in healthy condition. CONCLUSION: This case shows to surgeons that pancreatic pseudocyst cannot be managed strictly with one rule and prompt surgical treatment is mandatory in case of haemodinamic instability.

Solitary floating vena caval thrombus as a late recurrence of renal cell carcinoma.

We report an unusual case of solitary thrombus floating in the inferior vena cava (IVC) in a patient who underwent radical nephrectomy for a renal cell carcinoma (RCC) of the right kidney extended into the renal vein with no capsular and perinephric tissue invasion (pT3b). Twenty months after surgery, a routine computed tomography scan identified an intraluminal mass floating in the IVC. Cavotomy and thrombectomy with no caval resection were successfully performed. A review of the literature showed only three previous published cases of RCC recurring in the IVC only, with no local recurrence or distant metastases. We outline the possible etiology of these unusual and solitary recurrences in the IVC and we emphasize the need for a strict surveillance for all patients with RCC and especially for those with pT1b, pT2 and pT3 disease. An early diagnosis of this rare recurrence can permit an easy removal of the thrombus with no caval resection and graft replacement, making this disease potentially curable by surgery.