Adenoid cystic carcinoma of trachea treated with adjuvant hypofractionated tomotherapy. Case report and literature review

Adenoid cystic carcinoma, also called cylindroma, is the second most common histological type of tracheal malignancy but represents 1% of all respiratory tract cancers. We report a case of a 59-year-old patient submitted to an incomplete resection of the trachea and subsequently treated with adjuvant tomotherapy. There have been no reports in the literature regarding intensity-modulated radiation therapy with linac or tomotherapy systems in adenoid cystic carcinoma of the trachea. The present clinical case demonstrates the feasibility of adjuvant intensity-modulated radiation therapy techniques for optimizing the dose coverage of the tumor bed while sparing surrounding normal tissues. A dosimetric comparison between the tomotherapy plan and a 3-dimensional conformal radiotherapy plan is also reported. We demonstrate that tomotherapy permits an increase in the dose per fraction without important acute adverse effects. At 24 months' follow-up, our patient shows no evidence of disease with negative histological findings.     

Fractionated stereotactic radiation therapy for extracranial head and neck tumors

BACKGROUND: This study is to report the clinical experiences of fractionated stereotactic radiation therapy (FSRT) for extracranial head and neck tumors. METHODS AND MATERIALS: Between the period of July 1995 and November 1998, 48 patients with extracranial head and neck tumors were given FSRT as a boost and sole modality. Individualized treatment planning was performed using XKnife-3 system with relocatable Gill-Thomas-Cosman frame. In 24 patients, FSRT was applied as a boost technique following the 2-dimensional conventional external radiation therapy (ERT); in 24 patients FSRT was the sole radiotherapy modality. The primary diseases in the boost group consisted of nasopharynx cancer (19), lacrimal gland adenoid cystic carcinoma (3), orbital lymphoma (1), and skull-base recurrence of maxillary sinus adenoid cystic carcinoma (1). The primary diseases in the sole modality group consisted of recurrent nasopharynx cancer (12), orbital pseudotumor (4), skull-base recurrence of maxillary sinus, submandibular gland, and hypopharynx cancers (3), orbital rhabdomyosarcoma (2), orbital lymphoma (1), orbital metastasis of neuroblastoma (1), and nasal cavity melanoma (1). The fractionation schedule was to give 5 treatments per one week and the fractional doses were 2.0-3 Gy depending on the treatment aim and the FSRT volume. The FSRT doses varied depending on the nature of the primary diseases. RESULTS: The local tumor response in nasopharynx cancer patients was excellent compared to retrospective data without occurrence of unexpectedly severe complication. FSRT to other regions was well tolerated by the patients and resulted in good to excellent local tumor responses with no unacceptable side effects as expected by the authors. CONCLUSION: Based on the current observations, FSRT is a very effective and safe modality in the treatment of extracranial head and neck tumors.     

Adenoid cystic carcinoma of the lung: Response to tamoxifen after chemoradiation

Primary adenoid cystic carcinoma of the lung is a rare subtype of lung cancer with a prolonged natural history but is difficult to completely resect surgically due to its proximal location. Hence radiotherapy plays a role for local control of these cancers. The role of systemic therapies in the treatment of adenoid cystic carcinoma of the lung remains unclear. Isolated case reports have previously described the treatment of primary adenoid cystic carcinoma of the lung with tamoxifen, resulting in partial control. We report the first case of primary adenoid cystic carcinoma of the lung treated with primary chemoradiotherapy due to unresectable location of the tumor, and disease stabilization with symptomatic response to tamoxifen following tumor recurrence. There is a need for further studies to investigate the role of anti‐hormonal therapies in the treatment of adenoid cystic carcinoma of the lung.     

31例复发性腮腺腺样囊性癌临床分析

目的探讨腮腺腺样囊性癌诊断及复发癌治疗方法。方法对31例复发性腮腺腺样囊性癌作临床分析。结果局部切除或浅叶切除复发时间比全腮腺切除复发时间短。复发后采用手术 放疗比单纯手术的疗效好。颈淋巴结转移率低,本组为10.3%(3/29)。结论B超、CT及肿瘤穿刺细胞学检查有助术前诊断。全腮腺切除应视为常规,术后复发可采用扩大切除术,但应补充放疗。预防性颈淋巴结清除术不应视为常规。     

31例复发性腮腺腺样囊性癌临床分析

目的探讨腮腺腺样囊性癌诊断及复发癌治疗方法。方法对31例复发性腮腺腺样囊性癌作临床分析。结果局部切除或浅叶切除复发时间比全腮腺切除复发时间短。复发后采用手术＋放疗比单纯手术的疗效好。颈淋巴结转移率低，本组为10.3％（3／29）。结论B超、CT及肿瘤穿刺细胞学检查有助术前诊断。全腮腺切除应视为常规，术后复发可采用扩大切除术，但应补充放疗。预防性颈淋巴结清除术不应视为常规。     

Sinonasal adenoid cystic carcinoma: the M. D. Anderson Cancer Center experience

BACKGROUND: Adenoid cystic carcinoma of the sinonasal tract is a rare cancer that accounts for 10% of all malignancies at this site. The objective of the current study was to evaluate prognostic factors, treatment outcomes, recurrence patterns, and survival rates for sinonasal adenoid cystic carcinoma. METHODS: A retrospective chart review was performed at an academic tertiary referral center. Between 1990 and 2004, 105 patients were evaluated for adenoid cystic carcinoma of the sinonasal tract at a single institution. Demographics, presentation, anatomic site, Tumor, Lymph Node, Metastasis (TNM) classification, pathology, treatment, recurrences, and survival were evaluated. RESULTS: One hundred five patients with adenoid cystic carcinoma were evaluated, including 58 women and 47 men. Their median age was 50 years, and the mean follow-up was 47 months. The maxillary sinus (47%) and the nasal cavity (30%) were the most common primary tumor sites. The majority of patients presented with T3/T4 (76.7%), N0 (98%), M0 (97%) disease. Eighty-four percent of patients underwent surgery and received postoperative radiation as treatment for their primary disease. The local recurrence rate was 30%, and the distant metastases rate was 38%. The 5-year overall survival and disease specific survival rates were 62.9% and 70.9%, respectively. CONCLUSIONS: Adenoid cystic carcinoma of the paranasal sinuses is a rare disease, and the ideal treatment paradigm has yet to be defined. The current data suggested that surgical resection with postoperative radiation therapy offers durable local control and compares favorably with historic data. Although local recurrences develop in a significant percentage of patients, survival from this disease exceeds that of other sinonasal malignancies.     

Unresectable basaloid squamous cell carcinoma of the trachea treated with concurrent chemoradiotherapy: a case report with review of literature

Basaloid squamous cell carcinoma is an uncommon variant of squamous cell carcinoma of the trachea. We describe the case of an unresectable basaloid squamous cell carcinoma of the trachea treated with concurrent chemoradiotherapy up to a dose of 60 Gy in 33 fractions with weekly paclitaxel and carboplatin. The pathological recognition of basaloid squamous cell carcinoma and its distinction from adenoid cystic carcinoma of the trachea is important for its management. Combining systemic chemotherapy with locoregional radiation is a logical approach to treatment, especially for the basaloid squamous cell carcinoma of the trachea, given its tendency to metastasize early after definitive therapy.     

Primary Adenoid Cystic Carcinoma of the Skin with Multiple Local Recurrences

Primary adenoid cystic carcinoma (PACC) of the skin is a rare tumor with fewer than 70 cases studied in detail in the English literature. This type of tumor shows a prolonged course and a growth pattern usually manifested by multiple local recurrences and has a low potential for distant metastases. The most important modality for primary treatment is surgical resection followed by radiotherapy. We report a woman aged 43 years at the time of diagnosis, who presented with a slow-growing nodule in the right axilla without lymph node enlargement. A wide local excision was performed, and the histology revealed an adenoid cystic carcinoma. During the next 24 years, another four local recurrences were excised (the last one in 2015) and confirmed histologically to be adenoid cystic carcinoma. The patient was given 44 Gy of radiotherapy after the second surgery in 1996. PACC of the skin is a rare tumor with insufficient data concerning the efficacy of the surgical technique and chemotherapy and radiotherapy treatment, even more so in the case of multiple recurrences. After the last recurrence, the patient was offered an active follow-up based on the long tumor-free intervals in the past and because the site of the primary tumor allowed further surgical excisions in future recurrences.Key Words: Adenoid cystic carcinoma, Cylindroma, Skin     

涎腺腺样囊性癌血道转移的临床分析

目的：探讨腺样囊性癌血道转移问题,期望在临床上对此类病例的治疗引起重视。方法：对６例腺样囊性癌血道转移的病人进行回顾性分析。结果：腺样囊性癌因其生物性特征,术后容易发生局部复发和血道转移。结论：对此类病例强调首期完成根治性手术和进行综合治疗。密切随访,及时发现和治疗转移灶。     

Adenoid cystic carcinoma of the esophagus. Complete response to combination chemotherapy

A 55-year-old man had adenoid cystic carcinoma of the esophagus metastatic to the lungs and right supraclavicular fossa. He was treated with local radiation therapy to the esophagus and supraclavicular fossa, followed by combination chemotherapy with doxorubicin, mitomycin C, and 5-fluorouracil (5-FU). After a modest initial response, disease progression was noted in the pulmonary nodules. He was then treated with cisplatin, cyclophosphamide, vincristine, and doxorubicin. After two cycles of this regimen, there was complete regression of his pulmonary nodules, which was sustained for 5 months. A review of 44 literature cases of esophageal adenoid cystic carcinoma contrasted with adenoid cystic carcinoma of salivary gland origin indicated that the esophageal adenoid cystic carcinomas have a high tendency to metastasize (76% of cases) and a much poorer prognosis, with only 23% 1-year survival rate. It was concluded that esophageal adenoid carcinoma is clinopathologically distinct from the salivary gland variant, and that combination chemotherapy may be an effective treatment modality for this cancer.     

Multiple cutaneous metastases from a parotid adenoid cystic carcinoma

Cutaneous metastasis from salivary gland adenoid cystic carcinoma is extremely rare. We report a case of a 39-year-old man that presented multiple cutaneous metastases from a parotid salivary gland adenoid cystic carcinoma. The clinical, histopathological and immunohistochemical features are described and discussed. This case shows the importance of a detailed and periodical skin examination in patients treated for salivary gland adenoid cystic carcinoma.     

Salivary gland tumors of the oral cavity

Between 1961 and 1985, 62 patients with malignant salivary gland tumors of the oral cavity underwent surgery with curative intent at the University of California, Los Angeles (UCLA) Medical center. All patients had a minimum follow-up of 2 years. Fifty of 62 (81%) patients presented with T1-2 primary tumors. The tumors arose from the palate in 41/62 (66%) patients. The most common histologic type was adenoid cystic carcinoma comprising 34 of 62 (55%) cases. Radical resection was performed in 46 cases and wide local excision in 16 patients. Postoperative radiation therapy was used in 24 cases due to advanced stage and/or positive surgical margins. Results of treatment were analyzed by stage of disease, modes of treatment, histology, and surgical extent. Local control of small lesions reached 100% at 10 years with either radical resection alone or local excision. With residual tumor at the surgical margins, the incidence of local recurrence was 4/14 (29%) for those who received adjuvant radiation therapy and 5/10 (50%) for those who did not. The vast majority of mucoepidermoid carcinomas were early stage and low grade. These lesions had an excellent prognosis with a control rate of 100%. In contrast, there was a 29% (10/34) failure rate for adenoid cystic carcinoma. The 5-, 10-, and 15-year actuarial survivals for the whole group were 94%, 84%, and 73%, respectively. Our results indicate that for early stage disease, wide local excision may offer patients the chance to avoid the cosmetically and functionally debilitating effects of radical surgery without compromising treatment outcome. Adjuvant radiation therapy appears to reduce the local recurrence for those with residual tumor at the surgical margins.     

13例原发性颈段气管腺样囊性癌的手术治疗

国内外很少报道原发性颈段气管腺样囊性癌的外科治疗,本研究探讨13例该类患者外科治疗的有效性。     

食管腺样囊性癌22例报道并文献复习

目的 食管腺样囊性癌(adenoid cystic carcinoma,ACC)临床上罕见,文献多为个案报道或小样本病例,对本病缺乏系统了解.本研究回顾性分析22例食管ACC患者,分析其临床病理特征及诊疗策略,为临床提供参考.方法 分析河北医科大学第四医院2004-01-01-2013-12-31行根治性手术切除,且术后病理确诊为食管ACC患者的临床病理资料,采用Kaplan-Meier法进行生存分析.结果 食管ACC发病率男性多于女性,男女比例为4.5∶1.发病部位以胸中段最常见,为14例;其次是胸上段和胸下段,各4例.Ⅰ期19例,Ⅱ期2例,Ⅲ期1例.食管ACC的术前内镜诊断率较低,仅为27.3％(6/22),其中11例被误诊为鳞状细胞癌,4例误诊为腺癌,1例误诊为腺鳞癌.全组患者淋巴结转移率为9.1％(2/22),淋巴结转移度为0.9％(2/229),肺转移发生率为27.3％(6/22).全组患者1、3、5年生存率分别为90.9％、81.3％和65.1％,术后综合治疗与单纯手术相比生存率差异均无统计学意义,x2=0.054,P=0.816.结论 食管ACC发病男性多于女性,发病部位以胸中段最为多见,术前内镜诊断确诊率低.食管ACC总体预后较好,淋巴结转移并不常见,治疗失败以肺转移最为多见,术后是否需行综合治疗尚待进一步探索.     

The use of radiation therapy in the management of minor salivary gland tumors

Between 1964 and 1985, 52 patients were treated with curative intent by radiation therapy alone or in combination with surgery for malignant tumors of minor salivary gland origin. All patients had a minimum follow-up of 2 years, and 80% had a minimum follow-up of 5 years. Twenty-six (50%) were adenoid cystic carcinomas; the remaining histologies included adenocarcinoma, mucoepidermoid carcinoma, and malignant mixed tumors. The most common sites of origin were in the oral cavity/oropharynx (49%) and the nasal cavity or paranasal sinuses (40%). Twenty-seven patients (52%) presented with an advanced or unresectable stage (AJCC Stage III or IV, extensive bone or nerve invasion, or tumor greater than 5 cm). Treatment was highly individualized; 50% of the patients received radiation therapy alone, and 50% received combined treatment with either postoperative or preoperative radiation therapy. Early-stage minor salivary gland tumors were controlled equally well with radiation therapy alone or with a combined approach. For the advanced tumors, a combined approach yielded significantly superior absolute local control rates as compared with radiation therapy alone (10/13 vs. 2/13). For adenoid cystic carcinoma, the local control rate at 10 years was 45% (actuarial); the tumor was not controlled locally in any patients with advanced/unresectable stage who were treated with radiation therapy alone. The absolute local control rate was 75% for 4 early-stage tumors treated with radiation therapy alone and 60% for 5 advanced tumors treated with a combined approach. The average time to local recurrence was 67 months for adenoid cystic carcinoma. Severe complications of radiation therapy occurred in 11 (27%) of 40 evaluable patients, with unilateral blindness being the most common. Seven of 9 patients who became blind had unresectable disease with close proximity to or invasion of the orbit. A time-dose analysis is also presented.     

Multiple brain metastases: a rare manifestation of adenoid cystic carcinoma of the parotid gland

A rare case of multiple hematogenous brain metastases from adenoid cystic carcinoma of the parotid gland is reported. The patient had a parotid tumor that was treated ten years prior to the appearance of the brain metastases. Magnetic resonance imaging (MRI) and histological findings, as well as the radiation therapy response, of this tumor are presented.     

Tracheobronchial gland tumors.

BACKGROUND: Tracheal tumors are uncommon, making up only 0.2% of all respiratory malignancies in the United States. One consequence of this low incidence is that few centers accumulate meaningful experience. Another is the lack of awareness of effective therapy. Bronchial gland tumors demonstrate oncologic diversity and include benign, low-grade, and high-grade malignant tumors. METHODS: We reviewed the present knowledge of bronchial gland tumors of the trachea, carina, and bronchi, including the epidemiology, presentation, evaluation, tumor types, and treatment options. RESULTS: The malignant bronchial gland tumors, adenoid cystic carcinoma and mucoepidermoid carcinoma, are far more common than benign mucinous cystadenoma or pleomorphic adenoma. Complete resection of localized tumors has excellent long-term results in symptomatic benign tumors. The disease-free survival after resection of malignant tumors is limited by distant metastasis and regional disease, while local recurrence is uncommon. Postoperative mediastinal radiation is now accepted adjuvant therapy. Experience at our institute demonstrates a significant survival advantage for patients with complete resection compared to unresectable patients. CONCLUSIONS: Expanding knowledge of diagnostic evaluation and surgical therapy can improve the long-term survival of patients with tracheobronchial gland tumors.     

体部伽玛刀治疗头颈部腺样囊性癌多发肺转移瘤临床观察

 目的　观察体部伽玛刀治疗头颈部腺样囊性癌多发肺转移瘤的临床疗效。方法　对15例头颈部腺样囊性癌肺转移瘤患者共计137个病灶采用立体定向体部伽玛刀治疗。单次治疗病灶2～10灶,中位数5灶。以50 %剂量线为处方剂量线,单次照射剂量3～5 Gy,中位数4 Gy,1次／ d,5次／周,10～16次。肿瘤边缘总剂量30～52 Gy,中位数40 Gy。结果　137个病灶中,完全缓解58灶（42.3 %）,部分缓解64灶（46.7 %）,稳定15灶（10.9 %）,进展0灶,总有效率为89.1 %。治疗后1、2、3年局控率均为100.0 %。治疗后1、2、3、4、5年生存率分别为100.0 %、100.0 %、90.9 %、63.6 %、31.8 %。结论　体部伽玛刀治疗头颈部腺样囊腺癌多发肺转移瘤疗效较好,反应轻微,是一种安全有效的治疗手段。     

Partial remission of advanced adenoid cystic carcinoma obtained with adriamycin: a case report with a review of the literature.

Adenoid cystic carcinoma is relatively uncommon and often originates from the salivary glands. Although distant metastases develop rather frequently no satisfactory form of therapy has been reported. We achieved a partial remission with adriamycin in a patient with advanced metastases of adenoid cystic carcinoma. This case is described, and a short review of the literature, including papers on the treatment of advanced disease, is also given.     

Outcomes and prognostic variables in adenoid cystic carcinoma of the head and neck: a recent experience

PURPOSE: To analyze the recent experience of patients with adenoid cystic carcinoma treated with radiation therapy at Memorial Sloan-Kettering Cancer Center. METHODS AND MATERIALS: From 1990 to 2004, a total of 59 patients with a diagnosis of primary adenoid cystic carcinoma of the head and neck received radiation therapy at our institution. The subsite distribution was oral cavity, 28% (n = 17); paranasal sinuses, 22% (n = 13); parotid, 14% (n = 8); submandibular, 14% (n = 8); oropharynx, 10% (n = 6); sublingual, 3% (n = 2); nasopharynx, 3% (n = 2); and other, 5% (n = 3). T Stage distribution was T1, 34% (n = 20); T2, 19% (n = 11); T3, 14% (n = 8); and T4, 34% (n = 20). Twenty-nine percent of patients (n = 17) were treated with intensity-modulated radiation therapy; 25% (n =15), with three-dimensional conformal therapy, and the remainder, with conventional techniques. Ninety percent (n = 53) of patients received treatment including the base of skull. RESULTS: Median follow-up for surviving patients was 5.9 years. Five-year and 10-year rates of local control and distant metastases-free survival were 91%/81% and 81%/49%, respectively. Five-year and 10-year rates of disease-free and overall survival were 76%/40% and 87%/65%, respectively. On univariate analysis, stage T4 (p = 0.004) and gross/clinical nerve involvement (p = 0.002) were associated with decreased progression free survival, whereas stage T4 and lymph node involvement were associated with decreased overall survival (p = 0.046 and p < 0.001, respectively). CONCLUSIONS: Radiation therapy in combination with surgery produces excellent rates of local control, although distant metastases account for a high proportion of failures. Routine treatment to the base of skull reduces the significance of histologic perineural invasion, but major nerve involvement remains an adverse prognostic factor.