Merkel cell carcinoma of unknown primary site

Merkel cell carcinoma is an uncommon and aggressive primary neuroendocrine skin malignancy which mostly affects the extremities and the head and neck region of elderly patients. Merkel cell carcinoma occurs with increased frequency in sun-exposed areas, in individuals exposed to arsenic and in immunosuppressed patients. Many patients with Merkel cell carcinoma present with other malignancies, mainly skin cancers. Characteristic features are frequent recurrences and regional and distant metastases. Mortality rates range from 20 to 65 per cent. The mainstay of treatment is surgery, with wide local excision, and adjuvant radiotherapy is usually administered. Merkel cell carcinoma of unknown primary site is rare, and the majority of the few cases described have not been from head and neck areas. We present a case of Merkel cell carcinoma of unknown primary site, with upper neck and distant metastases.     

Incidence of epithelial neoplasms in the head and neck area in non-Hodgkin's lymphomas

There is an increased risk of malignancy in patients with genetically determined or acquired immunosuppression. The authors report a case of a 70-year-old patient with a lymphoplasmocytic immunocytoma who developed 19 squamous-cell carcinomas (SCC) and nine basal-cell carcinomas (BCC) over a three-year period. This was the reason to review 100 cases of malignant lymphomas for evidence of additional malignancies. Of these patients, 15% had one or more SCC or BCC in the head and neck area. The age range was 59 to 79 years (mean, 71.7 years) and the male:female ratio 11:4. One or more SCC arose in 93% of these patients, 36% developed an additional one or more BCC, and BCC alone occurred in 7%. The usual ratio of BCC:SCC is 10:1; in the authors' patients, by contrast, this ratio was 6:14. In 12 cases, SCC and BCC were located on the skin. The remaining cases of SCC developed in the oral mucosa, the tonsils and the hypopharynx. In 13 cases, low-grade malignant lymphomas were found and in two cases high-grade malignant lymphomas were found. The SCC were clinically aggressive. Thirty-six percent of the patients had recurrent lesions, 43% multiple neoplasms, and 50% metastases. Histologically, the SCC showed moderate to poor differentiation, a high degree of cell polymorphism and mitotic activity, and deep tissue infiltration. There are several explanations for the increased incidence of neoplasms in patients with immunodeficiency disorders. The surveillance function of the immune system may be impaired due to the disease itself or due to the treatment for immunosuppression. Immunosuppressive and cytotoxic agents are potential carcinogens.(ABSTRACT TRUNCATED AT 250 WORDS)     

Histological criteria and metastasis of squamous cell carcinoma of the pinna

INTRODUCTION: According to the guidelines for cancer of the skin in the head and neck region of the German association of Otolaryngology, Head and Neck surgery squamous cell carcinoma (SCC) of the pinna are classified by using the current TNM system of skin cancer. As soon as cartilage is infiltrated, irrespective of other criteria like tumour size, SCC of the thin skin of the pinna are classified as T4 category. As therapy considerably depends on the TNM stage a review of the prognostic value of cartilage infiltration as well as other histological criteria seems to be justified. METHODS: Medical records of all patients (n = 36) being operated for SCC of the pinna between August 1988 and January 2004 at our department were retrospectively analysed with regard to a statistical correlation of histological criteria, cervical lymph node metastases and prognosis. RESULTS: In 36 cases a histological re-evaluation could be performed on the original tumour samples (34 male symbol : 2 female symbol; average age was 76 years, with an age interval of 54 - 99 years). 26 SCC cases were smaller than 2 cm, 8 cases between 2 and 5 cm and 2 cases bigger than 5 cm of size. 36 % of SCC cases had infiltration of the auricular cartilage. Statistical analysis did not show a statistical correlation of either cartilage infiltration, tumour size bigger than 2 cm or 1 cm, tumour grading and tumour depth with regard to lymph node metastases. DISCUSSION: According to our results, cartilage infiltration as single criterion of inclusion into a T4 category should be analysed cautiously. The anatomical peculiarity of the pinna where cartilage lies directly beneath very thin skin should be taken into account. A survey of a bigger group of patients e. g. as a multicenter study would be desirable for such a rare malignancy.     

A comparative analysis of anterior versus posterior squamous cell carcinoma of the tongue: a 10-year review

INTRODUCTION: The study sought to compare and contrast squamous cell carcinoma (SCC) of the anterior mobile tongue with SCC of the tongue base, with emphasis on clinical presentation, management and outcome. METHODS: This was a retrospective, comparative analysis of patients treated for SCC of the tongue over a 10-year period. Cox's regression model was used to assess the effect of tumour site on survival. RESULTS: The study included 142 patients, of whom 86 were treated for SCC of the anterior tongue and 56 for tongue base lesions. Patients with carcinoma of the anterior tongue tended to present with a visible lump or ulceration of the tongue, whereas the majority of patients with tongue base SCC presented with pain. Sixty per cent of anterior tongue lesions were early stage (I or II) at initial presentation as compared with 21 per cent of tongue base lesions. CONCLUSION: Patients with anterior tongue lesions had a better prognosis, but this was not statistically significant when adjusted for stage.     

Neoadjuvant docetaxel and cisplatin chemotherapy followed by local irradiation is highly active on locoregionally advanced squamous cell carcinoma of the head and neck

The purpose of this study was to determine the treatment outcome of neoadjuvant docetaxel and cisplatin chemotherapy followed by local radiotherapy for chemotherapy-na?ve patients with locoregionally advanced squamous cell carcinoma of the head and neck. Thirty-seven patients with stage III or IV squamous cell carcinoma of the head and neck who received docetaxel and cisplatin regimen for a maximum of three cycles followed by radiation therapy were enrolled in this study. The overall response rate to the regimen was 91.9 per cent (34 of 37) (the complete remission rate was 48.6 per cent). The median time to treatment failure was 38 months (95 per cent confidence interval, 15-61 months). The four year estimated overall survival rates were 85.1 per cent. The most frequent moderate-to-severe toxicity was grade 3-4 neutropenia. The most common acute non-haematologic toxicities included anorexia, nausea and asthenia. Neoadjuvant docetaxel and cisplatin chemotherapy followed by radiotherapy is a feasible treatment strategy for patients with locoregionally advanced squamous cell carcinoma of the head and neck.     

Coexistence of laryngeal squamous cell carcinoma and non-Hodgkin's lymphoma with nasopharyngeal involvement

Laryngeal squamous cell carcinoma (SCC) is one of the most frequent malignancies in the head and neck region. The risk of multiple malignancies is reported as 2-11 per cent and most of the second primaries are SCCs. Lymphogenic tumours as second primaries are extremely rare. In this paper, we report a case of laryngeal SCC with synchronous non-Hodgkin's lymphoma and review the literature on the clinical and histopathological aspects of these malignancies.     

Skin and eye: uncommon sites of distant metastasis from tongue base squamous cell carcinoma

The frequency of non-lymphatic distant metastases from primary head and neck squamous cell carcinoma (SCC) appears to be higher than previously presumed. The general conditions of the affected patients, who usually also present with locoregional recurrences, are so impaired as to limit the use of available methods for diagnosing the distant spread of head and neck SCC. The incidence reported in autopsy studies is approximately three to four times higher than that in clinical studies. Lung metastases from head and neck SCC are most common, followed by metastases to bone and liver. Metastases to the skin are unusual. Secondary ocular localizations of head and neck SCC are exceedingly rare. We report the first case of synchronous intraocular (involving the choroid and vitreous body) and cutaneous metastases from a recurrent tongue base SCC in a 64-year-old woman who had undergone radiotherapy, bilateral neck dissection and chemotherapy. Cytological evaluation of vitreous aspirate and histological diagnosis of the skin lesion were performed < 1 month before the patient's death. Skin metastases occur in 1-4% of patients with diagnosed head and neck SCC and are usually associated with advanced or recurrent disease. To the best of our knowledge, fewer than 10 cases of ocular metastases from head and neck SCC have been reported. The average survival time after diagnosis of ocular or skin metastases from head and neck SCC is 7 months. Treatment for eye and skin metastases is palliative and rarely alters patient outcome.     

头颈部转移性透明细胞癌5例并文献复习

目的 探讨泌尿系统透明细胞癌转移至头颈的部位和治疗方法.方法 总结我院2011年至2018年收治的具有完整临床资料的5例头颈部转移性透明细胞癌,分析原发疾病、原发病治疗方法、转移时间、转移部位、转移灶治疗方法、预后等临床资料.结果 5例透明细胞癌原发灶位于肾脏4例、膀胱1例,原发灶均行根治性手术.转移时间为原发灶术后1...     

Squamous cell carcinoma of the hypopharynx treated with surgery and radiotherapy

A series of squamous cell carcinomas (SCC) of the hypopharynx treated with combined surgery and radiotherapy is presented to highlight the results of treatment at an early stage of disease. A retrospective mono-institutional analysis was performed on 153 previously untreated patients with SCC of the hypopharynx, seen between 1980 and 1995 at our institution. Univariate and multivariate analyses were performed using the Cox proportional hazard model. The overall five-year specific, and non-specific, disease survival rates were 68 per cent (95 per cent confidence interval, CI: 60-77) and 47 per cent (95 per cent CI: 39-56), respectively. Compared with other series, this study is characterized by treatment at an earlier stage, better prognosis, and a higher number of multiple malignancies. Twenty-two per cent of hypopharyngeal SCCs were diagnosed during the staging procedures for a different head and neck SCC and 14 per cent during the follow-up for a previous tumour. Multivariate survival analysis of clinical and pathological factors confirmed the clinical class of tumour (T) and node (N) and the nodal capsular rupture as prognosticators of disease.     

Radiological staging of the chest and abdomen in head and neck squamous cell carcinoma--are computed tomography and ultrasound necessary?

The need for, and choice of, radiological staging investigations for distant metastases in the management of head and neck squamous cell carcinoma is a contentious issue. To address this problem a retrospective audit of routine computerized tomography (CT) and ultrasound scanning of the chest and abdomen respectively was undertaken. The records of 103 patients who, over a six and a half year period, underwent major surgery for head and neck squamous cell carcinoma were reviewed. A total of 57 patients (59 per cent) had CT scanning of the chest of whom two were identified as having synchronous tumours. In both cases, the lesions were identified on chest X-ray prior to scanning. Seventy patients (68 per cent) had routine ultrasound scanning of the abdomen. In none of these was metastatic disease identified. As a result of the audit findings routine CT and ultrasound scanning of the chest and abdomen has been discontinued.     

Presentation,course, and outcome of head and neck skin cancer in african americans: A case-control study

Background: Several reports have shown that the presentation, course, and outcome of skin cancer is altered in African Americans. Subset data from these studies suggest that the course of head and neck skin cancer may be different from that occurring in other sites. However, very few studies have specifically investigated skin cancer involving the head and neck region in African-American patients. Methods: Retrospective case-control study including 215 patients with skin cancer (squamous cell carcinoma [SCC], basal cell carcinoma [BCC], malignant melanoma, and adnexal tumors) presenting to a tertiary care institution over a 9.5-year period. Cases were defined as African Americans with skin cancer, and the control group included white and Latin-American patients with skin cancer. Results: Skin cancer occurred in the head and neck region in 135 cases (62%). However, head and neck involvement was less common in African-American patients (44%) than the control group (76%; P < .001). The anatomic distribution of head and neck skin lesions was similar between the groups, with nasal and scalp skin most often involved. In the head and neck region, the ratio of BCC to SCC (4:1) was similar among all groups. In contrast, in non–sun-exposed regions, the ratio was 1:8.5 for African-American patients compared with 1:1 for the control group (P < .001). The overall distribution of malignant melanoma was not influenced by sun exposure in either groups. The study groups were similar in gender distribution, primary treatment modality, rates of positive margins, and development of second skin cancers. Although African Americans presented with more advanced lesions (P < .001), their disease-free interval was similar to the control group. Only the margin status was a significant predictor of disease-free survival by multivariate analysis, with a relative risk of 1.68 (95% CI: 1.58–18.24) Conclusions: Head and neck skin cancer is similar with regard to presentation and distribution in patients of all skin types. Moreover, in contrast to previous reports, the course of head and neck skin cancer may be less aggressive in African Americans, if appropriate treatment is provided. This report suggests that differences in skin cancer in African Americans reported in the literature reflect cancer occurring in non–sun-exposed regions.     

Second primary squamous cell carcinoma arising in cutaneous flap reconstructions of two head and neck cancer patients

Early complications of myocutaneous flap transfers following surgical eradication of head and neck tumors have been extensively described. However, knowledge concerning long-term complications of these techniques remains limited. We report the cases of two patients with a prior history of squamous cell carcinoma of the head and neck (HNSCC), who developed a second primary SCC on the cutaneous surface of their flaps, years after reconstruction. Interestingly, it seems that the well-known risk of a second primary SCC in patients with previous head and neck carcinoma also applies to foreign tissues implanted within the area at risk. Given the important expansion of these interventions, this type of complication may become more frequent in the future. Therefore, long-term follow-up of patients previously treated for HNSCC not only requires careful evaluation of the normal mucosa of the upper aero-digestive tract, but also of the cutaneous surface of the flap used for reconstruction.     

Basaloid squamous cell carcinoma of the larynx

Basaloid squamous cell carcinoma (BSCC) is a rare tumor with distinct morphological and biological features that differentiate it from the common form of squamous cell carcinoma (SCC) in the head and neck region. It is mostly seen in the supraglottic larynx, hypopharynx and a base of the tongue. We present two cases of BSCC of the larynx; both being transglottic tumors. Both of the patients underwent primary surgery including bilateral neck dissections. None of the patients had cervical metastases at histopathological examination. Both patients received radiotherapy after surgery. They were alive and free of disease at 24 and 27 months, respectively.     

Squamous cell carcinoma in the immunosuppressed patient: Fanconi's anemia.

The association of immunosuppression and head and neck cancer is supported by numerous reports demonstrating impaired cell-mediated immunity, depressed T-cell function, decreased lymphocyte responsiveness, and elevated circulating immune complexes. Fanconi's anemia (FA) is a rare autosomal recessive syndrome characterized by progressive pancytopenia, skeletal abnormalities, hyperpigmentation, and other congenital anomalies. Increased chromosomal instability and defective DNA repair have been uniform findings. Several reports suggest associated immune deficiencies. There is an increased frequency of leukemia, hepatocellular carcinoma, and squamous cell carcinoma (SCC), including six cases of head and neck SCC. We reported a young girl with FA who developed SCC of the tongue. Initial studies suggest low lymphocyte counts, but normal lymphocyte responsiveness. More precise characterization of the immune system defects in malignancy prone, genetically determined syndromes may provide clues for the diagnosis and treatment of patients with the more usual but more variable risk factors for SCC of the head and neck.     

Lysis of squamous cell carcinoma via antibody-dependent cellular cytotoxicity

We have developed a system in which antibody-dependent cellular cytotoxicity (ADCC) can be consistently demonstrated against squamous cell carcinoma (SCC) of the head and neck. Serum samples from patients with pemphigus vulgaris (PV) provided antibodies for ADCC. The effector cells were lymphocytes from the peripheral blood of healthy human donors. The SCC cell lines served as targets. Lysis of SCC, as measured by 51Cr release, was significantly enhanced by the presence of serum from patients with PV, but not by healthy human serum. Serum alone produced no target cell killing. Nonepithelial cell lines were not affected by the presence of PV antibodies. The results demonstrate that SCC is susceptible to ADCC. Thus, tumor-specific antibodies may have a role in the treatment of cancer of the head and neck. This system can serve as a positive control for further testing of ADCC against SCC.     

Therapeutic effect of Nivolumab for advanced / recurrent temporal bone squamous cell carcinoma

ObjectiveThe immune checkpoint inhibitor Nivolumab was approved for the treatment of platinum-refractory head and neck squamous cell carcinoma (SCC), expanding the treatment options for recurrent or advanced head and neck SCC. However, since temporal bone squamous cell carcinoma (TB-SCC) is very rare cancer, the effectiveness of Nivolumab remains unclear. We investigated the effects of Nivolumab for TB-SCC.MethodChart information was collected for all patients who underwent the first administration of Nivolumab for recurrent or residual TB-SCC in our hospital between September 2017 and December 2019. Tumor staging followed the modified Pittsburgh classification. Changes in the tumor burden and survival outcome were examined.ResultsWe examined 9 patients with recurrent or residual TB-SCC who started administration of Nivolumab. In these cases, recurrent or residual SCC was observed after chemotherapy and/or chemoradiotherapy including platinum. The duration of Nivolumab was 2–54 weeks (median 20.0 weeks). The evaluation of the therapeutic effect according to the RECIST method showed partial response in 1 case, stable disease in 2 cases, progressive disease in 4 cases, and size unevaluated in 2 case. Although the number of cases was small, comparing with 5 cases without Nivolumab, these cases showed longer overall survival (1-year OS 33.3% vs 20.0%).ConclusionWe used Nivolumab as palliative chemotherapy in 9 patients with recurrent/residual TB-SCC, and we were able to obtain a certain therapeutic effect on TB-SCC as well as other head and neck SCC.     

Basosquamöses Karzinom der Haut

Background. Basosquamous carcinoma (BSC) is a rare malignancy with specific histopathological features of both basal cell (BCC) and squamous cell carcinoma (SCC). Therefore, the histological diagnosis is challenging. Due to its low incidence there is no consensus on the surgical management of BSC. Patients and methods. We describe the (immunohistological) diagnostic and therapeutic aspects of nine cases (8♂:1♀, on average 68.6 years of age (range: 47–81)) of BSC. Of these, seven were located on the pinna, one on the forehead and zygomatic region and one in the retroauricular region. Results. Immunochemical staining for epithelial membrane antigen was negative (apart from the typical areas of epithelial pearl formation) and BerEP4 was positive in all cases. Therapy consisted of partial removal of the pinna in four, and total removal in three cases. One patient was treated by partial removal of the auricle with superficial parotidectomy and ipsilateral neck dissection. In the case of the carcinoma on the forehead, a local excision was performed. The median follow-up was 45 months. One patient had a local recurrence. Conclusions. The histological diagnosis of BSC is confirmed by the use of EMA and BerEP4 immunohistological staining. Clinically, BSC is a rare, aggressive skin tumor. Despite the histological similarity to basal cell carcinoma, BSC has an imminent risk of metastasizing. Hence, therapy should be similar to that for SCC, taking into consideration the age and general state of the usually elderly patient.     

Squamous cell carcinoma of the nasal vestibule.

We reviewed 30 patients with squamous cell carcinoma of the nasal vestibule to present our experience of their management and to evaluate the prognostic factors that may influence their outcome. For T1 lesions radiotherapy remains the treatment of choice, because of the superior cosmetic result. Fifteen (68 per cent) out of 22 patients with T2 lesions were treated with primary radiotherapy. Surgery however, was eventually required in 16 (72 per cent) out of 22 patients, either as a primary treatment (seven patients) or as salvage surgery (nine patients). For the larger T3 lesions the treatment of choice is surgery followed by post-operative radiotherapy where appropriate. Regional nodal metastases at the time of presentation were a significant indicator of local and regional recurrence and of prognosis. The cause specific five-year survival for patients with an uninvolved neck (22 patients) was 100 per cent compared with 38 per cent for patients with nodal metastases at presentation (eight patients). Elective treatment for uninvolved regional nodes is not considered necessary.     

Occurrence and recurrence of basal cell carcinoma of the head and neck in Negroid and albinoid Africans

A study of sixty patients with basal cell carcinoma of the head and neck region carried out over a six-year period (1979-1985) is hereby presented. Sixteen (26.72 per cent) were albinos and 44 (73.28 per cent) negroids. Forty-eight (80 per cent) were outdoor workers. The negroid patients presented between the 3rd and 4th decades while the albinos presented a decade earlier. The commonest site involved in the head and neck was the forehead. The midface showed the highest recurrence rate in both groups, even after adequate excision. The frequency of recurrence in tumours presenting with a size of 2-5 cm. diameter was significantly higher in the albinos than in the negroid (P less than 0.05), whereas, with tumours of a size larger than 5 cm., there was no statistically significant difference between the albino and the negroid. However, the overall recurrence rate was significantly higher in the albinos (P less than 0.005). The mortality among the albinos was 25 per cent while there were no deaths in the negroid Africans.     

Head and neck basal cell carcinoma: treatment using a 2-mm clinical excision margin

Treatment of basal cell carcinoma (BCC) should completely remove the tumour whilst preserving the maximum amount of normal surrounding skin. Therefore, treatment is a compromise between safe excision margins and obtaining a satisfactory cosmetic result. We report the results of a prospective study of 63 patients who underwent excision of well-demarcated BCCs in the head and neck region. The surgical excision margin used was 2 mm. Histological assessment confirmed complete excision in 95% and there was no evidence of recurrence of the BCC over a 24-month follow-up period in all patients. We propose that a clinical excision margin of 2 mm is adequate for treatment of simple, well demarcated BCCs arising in the head and neck.