肾原发性大细胞神经内分泌癌伴高级别尿路上皮癌1例报道并文献复习

目的:探讨肾脏大细胞神经内分泌癌的临床病理特征、诊断和鉴别诊断。方法:分析1例肾原发性大细胞神经内分泌癌伴高级别尿路上皮癌患者的临床表现,对标本进行组织学观察,免疫组化染色,并复习相关文献。结果:患者为63岁男性,因间歇性血尿就诊。巨检:右肾中下部及肾盂处见一6cm×5cm×5cm肿块,切面灰白色,大部分实性质地中等、下极肾盂处部分呈乳头样质脆。镜检:实质性区域瘤细胞呈巢状、小梁状或条索状排列,片状坏死,瘤细胞Syn、ChgA和CD56均为阳性,Ki-67增殖指数达40%;乳头样结构处呈典型的尿路上皮乳头状癌图像。癌肿侵犯肾门脂肪组织伴肾门淋巴结转移。结论:肾原发性大细胞神经内分泌癌是罕见肿瘤,伴尿路上皮癌更罕见,具有高侵袭性,转归预后差,诊断主要依据病理形态学检查和免疫组织化学。     

A case report of primary small cell carcinoma of the breast and review of the literature

Primary small cell carcinoma (SCC) of the breast, an exceedingly rare and aggressive tumor, is often characterized by rapid progression and poor prognosis. We report a case of primary SCC of the breast that was diagnosed through pathologic and immunohistochemical examinations. Computed tomography (CT) scans failed to reveal a non-mammary primary site. Due to the scant number of relevant case summaries, this type of tumor is proved to be a diagnostic and therapeutic challenge. Therefore, we also reviewed relevant literature to share expertise in diagnosis, clinicopathologic characteristics, treatment, and prognosis of this type of tumor. Future studies with more cases are required to define more appropriate treatment indications for this disease.     

A pilot study of adjuvant chemotherapy with irinotecan and cisplatin for completely resected high-grade pulmonary neuroendocrine carcinoma (large cell neuroendocrine carcinoma and small cell lung cancer)

Background

Large cell neuroendocrine carcinoma (LCNEC) and small cell lung cancer (SCLC) are recognized as high-grade neuroendocrine carcinomas (HGNEC) of the lung. In patients with completely resected HGNEC, platinum-based adjuvant chemotherapy may be considered. However, the optimum chemotherapy regimen has not been determined. We conducted a multicenter single-arm phase II trial to evaluate irinotecan and cisplatin in postoperative adjuvant chemotherapy for HGNEC patients.

Patients and methods

Patients with completely resected stage I–IIIA HGNEC received four cycles of irinotecan (60 mg/m², day 1, 8, 15) plus cisplatin (60 mg/m², day 1). This regimen was repeated every 4 weeks. The primary endpoint was the rate of completion of chemotherapy (defined as having undergone three or four cycles), and secondary endpoints were the rate of 3-year relapse-free survival (RFS), rate of 3-year survival and toxicities.

Results

Forty patients were enrolled between September 2007 and April 2010. Patients’ characteristics were: median age (range) 65 [45–73] years; male 85%; ECOG-PS 1 60%; LCNEC 57% and SCLC 43%; stage IA/IB/IIB/IIIA 32/35/8/5%; 95% received lobectomy. The rate of completion of chemotherapy was 83% (90% C.I.; 71–90%). The rate of overall survival at 3 years was estimated at 81%, and that of RFS at 3 years was 74%. The rates of overall survival and RFS at 3 years were 86 and 74% among 23 LCNEC patients, and 74 and 76% among 17 SCLC patients, respectively. Nineteen patients (48%) experienced grade 3 or 4 neutropenia, but only five patients (13%) developed febrile neutropenia. Two patients (5%) developed grade 3 diarrhea, and four patients (10%) had grade 3 nausea. No treatment-related deaths were observed in this study. All 40 specimens were also diagnosed as HGNEC by central pathological review.

Conclusions

The combination of irinotecan and cisplatin as postoperative adjuvant chemotherapy was feasible and possibly efficacious for resected HGNEC.     

Small cell carcinoma of the gallbladder: Report of two cases

Two Taiwanese patients with gallbladder small cell carcinoma are reported. One is a 79 year-old male, the other, a 86 year-old female. They both presented with the symptom/signs of acute cholecystitis and underwent cholecystectomy. An intramural mass in the gallbladder neck region was found in the first patient, while the second patient had a transmural indurated tumor in the gallbladder body with extension to the neck region. Characteristic histological and immunohistochemical features of small cell carcinoma were present in both, and electron dense neurosecretory granules were identified in the second. To our knowledge, the second patient is the oldest ever reported. The first patient received chemotherapy directed toward the initial erroneous diagnosis of non-Hodgkin s lymphoma and developed liver metastasis in two months. The second patient did not receive chemotherapy due to her poor general condition and local recurrence occurred in six weeks. Both passed away three and five months after surgery, respectively.     

Progress in Diagnosis and Treatment of Small Cell Carcinoma of the Cervix

Small cell carcinoma of the cervix(SCCC)belongs to the neuroendocrine carcinomas,and it is a rare gynecological tumor of high-potential malignancy.It has a poorer prognosis compared to cervical squamous cancer or adenocarcinoma,and the therapeutic regimen of the disease differs.Diagnosis is based on pathomorphological characteristics,i.e.,the small and round cancer cel s(oat cel)which are uniform in shape and size,with the immunohistochemical marker helpful for diagnosis.Combined therapy is first recommended.Postoperative chemotherapy with platinum/etoposide (PE),vincristine/adriamycin/cyclophosphamide(VAC)and taxel/carboplatin (TP)can markedly improve the prognosis of early SCCC patients.     

宫颈小细胞癌的研究进展

目的:通过文献复习探讨宫颈小细胞癌(small cell carcinoma of the cervix,SCCC)的临床病理特点、生物学行为、治疗及预后情况。方法:应用检索Meta Med及CHKD期刊全文数据库检索系统,以"宫颈恶性肿瘤、小细胞癌和神经内分泌癌"为关键词,分别或联合检索1998-01-2008-06的相关文献80篇。纳入标准:1)组织发生;2)临床病理特点;3)化疗、放疗和综合治疗疗效;4)预后分析。根据纳入标准,精选59篇文献,最后纳入分析28篇文献。结果:SCCC是一种罕见的宫颈原发恶性肿瘤,与普通的子宫颈癌相比,SCCC具有高度侵袭性,常早期发生远处转移和局部复发,预后差。正确的临床诊断需联合光镜、电镜和免疫组化检查。因发病率低和各中心累积的病例数有限,目前尚无规范的治疗方法。手术是治疗早期SCCC的重要手段,同步放化疗可用于治疗晚期SCCC患者,联合化疗和放疗可能改善预后。临床期别是决定预后的重要因素。结论:SCCC预后差,强调早期诊断和综合治疗,由于SCCC罕见,需长时间才能积累一定数量的患者资料,建议通过多中心协作以探求最佳的治疗方案。     

Primary clear cell carcinoma of the nasopharynx： a case report

Primary dear cell carcinoma of the nasopharynx is a rare and locally invasive minor salivary gland neoplasm, only two cases have been reported yet in the literature. Now a 57-year man, with primary nasopharyngeal clear carcinoma,received radiotherapy and chemotherapy. After treatment, the mass of nasopharynx visibly reduced. Follow-up two months and repeated MRI, CT, abdominal B-ultrasonograpy, electronic nasopharyngoscopy, no tumor recurrence or metastasis.     

肺外小细胞癌65例临床观察及分析

经回顾性调查研究发现，该院１９５８～１９９４年间诊治的６５例肺外小细胞癌中，食管原发小细胞癌最为常见，其首发症状因原发部位不同而各异。５７例有明确预后的患者中，手术组中位生存期１０个月，手术＋化防组中位生存期２３．５个月，局限期患者２年生存率３２．４％，５年生存率８．８％，广泛期患者２年生存率４．３％，５年生存率为０。研究提示：治疗该病除早期切除外，给予系统、足量的化疗将有助于提高患者生存率。     

Silent advanced large cell neuroendocrine carcinoma with synchronous adenocarcinoma of the colon: A case report

BACKGROUNDLarge cell neuroendocrine carcinoma (LCNEC) accounts for about 0.25% of colorectal cancer patients. Furthermore, synchronous LCNEC and adenocarcinoma coexistence in the colon is very rare. LCNEC are usually aggressive and have a poor prognosis. Usually, colorectal LCNEC patients complain of abdominal symptoms such as pain, diarrhea or hematochezia because it is often diagnosed as an advanced disease that accompanies metastatic lesions.CASE SUMMARYWe describe a case of relatively asymptomatic synchronous LCNEC and colon adenocarcinoma. A 62-year-old male patient visited our hospital due to anemia detected by a local health check-up. He did not complain of melena, hematochezia or abdominal pain. Physical examination was unremarkable and his abdomen was soft, nontender and nondistended with no palpable mass. Laboratory tests revealed anemia with hemoglobin 5.1 g/dL. Colonoscopy revealed an ulcerofungating lesion in the ascending colon and about a 1.5 cm-sized large sessile polyp in the sigmoid colon. Endoscopic biopsy of the ascending colon lesion revealed the ulcerofungating mass that was LCNEC and endoscopic mucosal resection at the sigmoid colon lesion showed a large polypoid lesion that was adenocarcinoma. Multiple liver, lung, bone and lymph nodes metastasis was found on chest/abdominal computed tomography and positron emission tomography. The patient was diagnosed with advanced colorectal LCNEC with liver, lung, bone and lymph node metastasis (stage IV) and synchronous colonic adenocarcinoma metastasis. In this case, no specific symptom except anemia was observed despite the multiple metastases. The patient refused systemic chemotherapy and was discharged after transfusion.CONCLUSIONWe report a case of silent LCNEC of the colon despite the advanced state and synchronous adenocarcinoma.     

Metastasis of esophageal small cell carcinoma to the appendix: A case report and literature review

Primary small cell carcinoma (SCC) of the esophagus is characterized by high malignancy with a tendency to metastasize early through lymph and blood circulation. Metastasis of esophageal SCC frequently occurs to distant organs such as liver and lung. However, few cases of appendiceal metastasis have been reported. This paper first presents a pathologically confirmed case with metastasis of esophageal SCC to the appendix. This particular case highlights the importance of pathological diagnosis and provides new evidence of appendiceal metastasis from esophageal SCC.     

Merkel cell carcinoma: report of two cases and clinical considerations.

We present the clinical and pathological features of two cases of facial Merkel cell carcinoma (MCC) and a critical reappraisal of the literature on this subject. Among patients with this presentation of a rare neoplasm, the disease is very often localized (local or locoregional growth, without distant metastases). Radiotherapy seems to be highly effective in obtaining local control and possibly cure, even when used as the sole treatment method; prompt regression of the neoplastic masses has been observed in both the cases we treated. One of them, and many of those reported in the literature and treated with radiotherapy alone or combined with surgery, achieved long-term survival.     

Selective radiotherapy for the treatment of head and neck Merkel cell carcinoma

BACKGROUND.

The role of radiotherapy (RT) in the management of Merkel cell carcinoma (MCC) is controversial. The authors of this report evaluated the rates and patterns of failure in a selected group of patients who underwent RT for MCC of the head and neck (HN).

METHODS.

The records of 145 consecutive patients with MCC of the HN who presented to the authors' institution between 1988 and 2009 were reviewed. Only patients who received RT at the institution were included. The cumulative incidence of locoregional failure (LRF), distant metastatic failure (DMF), disease progression (DP) and disease‐specific death (DSD) were estimated with death as a competing risk.

RESULTS.

Forty‐eight patients were identified. The median follow‐up was 51 months (range, 6‐220 months) for living patients. LRF developed in 5 patients (10%), and those patients had a median time to recurrence of 3 months. Two of the 5 LRFs were local and developed at the edge of the treatment field; the remaining 3 LRFs were in lymph nodes and occurred outside the treatment field. DMF developed in 12 patients (25%). The estimated 5‐year cumulative incidences of LRF, DP, and DSD were 10%, 30%, and 21%, respectively. Acute toxicities included 5 episodes (10%) of grade 3 dermatitis and 1 episode (2%) of grade 3 mucositis.

CONCLUSIONS.

The authors report a site‐specific series of patients with HN MCC who received RT. In this group of patients with adverse features, RT was well tolerated, and LRF was low. The propensity for MCC to recur at the edge of the treatment field suggests that generous margins are appropriate when RT is administered. Cancer 2012. © 2011 American Cancer Society.     

Metastatic cutaneous squamous cell carcinoma to parotid nodes: the role of bolus with adjuvant radiotherapy

Introduction: Information regarding the addition of tissue equivalent bolus to adjuvant radiotherapy (RT) for intra‐parotid metastatic head and neck cutaneous squamous cell carcinoma is lacking. This study aimed to evaluate the effect of bolus versus no bolus on the patterns of regional and distant recurrence, regional control (RC), cancer‐specific survival (CSS), overall survival, RT toxicity and RT interruption. Methods: A retrospective study was performed on consecutive patients diagnosed between 1994 and 2008 with metastatic head and neck cutaneous squamous cell carcinoma who were treated with parotidectomy ± selective neck dissection and adjuvant RT ± parotid bolus. Results: Seventy‐five patients were identified: 64 males and 11 females, with median age of 79 years (range 40–96) of which 39 had bolus during RT. Median follow up was 48 months (range 4–177). There were 23 regional recurrences – 14 dermal, six dermal + nodal and three isolated nodal – and only two systemic recurrences. Nine patients had RT interruption >6 days due to acute skin toxicity. Bolus was associated with increased grade ≥3 radiation dermatitis (P = 0.02). RT interruption >6 days was significantly associated with inferior RC and hazard ratio, 2.83 (95% confidence interval: 1.04–7.71, P = 0.042). Lympho‐vascular space invasion, positive margins and nodes >2 cm were adversely significant on CSS multivariate analysis. RC, CSS and overall survival at 5 years were 67, 66 and 52%, respectively. Conclusions: Dermal involvement dominated the pattern of regional recurrence. Bolus was associated with significantly worse skin reaction. Bolus use was not associated with a significant overall benefit on RC. This analysis does not support the use of bolus as applied in this cohort.     

The recurrence and survival of oral squamous cell carcinoma: a report of 275 cases

Oral squamous cell carcinoma （OSCC） is a common malignant tumor of the head and neck, and recurrence is an important prognostic factor in patients with OSCC. We explored the factors associated with recurrence of OSCC and analyzed the survival of patients after recurrence. Clinicopathologic and follow-up data of 275 patients with OSCC treated by surgery in the Cancer Institute and Hospital of Tianjin Medical University between 2002 and 2006 were analyzed. Recurrence factors were analyzed with Chi- square or Fisher＇s exact test and multivariate analysis. The prognosis of patients after recurrence was analyzed with the Kaplan-Meier method and log-rank test. The recurrence rate was 32.7%. The recurrence time ranged from 2 to 96 months, with a median of 14 months. Univariate analysis showed that T stage, degree of differentiation, pN stage, flap application, resection margin, and lymphovascular invasion were factors of recurrence （P 〈 0.05）. Multivariate analysis showed that T stage, degree of differentiation, and pN stage were independent factors of recurrence （P 〈 0.001）. The differences in gender, age, tumor site, region of lymph node metastasis, and perineural invasion between the recurrence and non-recurrence groups were not significant （P 〉 0.05）. Kaplan-Meier and log-rank tests showed that the 2- and 5-year survival rates were significantly lower in the recurrence group than in non-recurrence group （67.6% vs. 88.0%, 31.8% vs. 79.9%, P 〈 0.001）. Therefore, to improve prognosis, we recommend extended local excision, flap, radical neck dissection, and adjuvant chemoradiotherapy for patients more likely to undergo recurrence.     

Prognostic factors in FIGO stage IB-IIA small cell neuroendocrine carcinoma of the uterine cervix treated surgically: results of a multi-center retrospective Korean study.

BACKGROUND: To determine the clinical and pathologic prognostic factors in surgically treated patients with International Federation of Gynecology and Obstetrics (FIGO) stage IB-IIA small cell neuroendocrine carcinoma of the uterine cervix (SCNEC). PATIENTS AND METHODS: We retrospectively reviewed a total of 68 patients with FIGO stage IB-IIA SCNEC surgically treated from January 1997 to December 2003 in Korea. RESULTS: Of the 68 patients, 43 had FIGO stage IB1 SCNEC, 15 had stage IB2, and 10 had stage IIA. Seven were treated with radical surgery alone; 11 with neoadjuvant chemotherapy (NACT) followed by radical surgery; 24 with radical surgery followed by adjuvant chemotherapy; and 26 with radical surgery followed by adjuvant radiation or chemoradiation. After a median follow-up of 44 months (range, 6-113 months), the 2-year and 5-year survival rates for all patients were 64.6% and 46.6%, respectively. Univariate and multivariate analysis showed that FIGO stage was predictive of poor prognosis. Patients who received NACT showed poorer prognosis than those who did not receive NACT. Adjuvant chemoradiation did not improve survival compared with adjuvant chemotherapy alone. CONCLUSIONS: FIGO stage may act as a surrogate for factors prognostic of survival. Primary radical surgery followed by adjuvant chemotherapy is the preferred treatment modality for patients with early stage SCNEC.     

Urinary bladder malignant paraganglioma with vertebral metastasis:a case report with literature review

Paraganglioma is a rare neuroendocrine neoplasm observed in patients of all ages, with an estimated incidence of 3/1,000,000 population. It has long been recognized that some cases are familial. The majority of these tumors are benign, and the only absolute criterion for malignancy is the presence of metastases at sites where chromaffin tissue is not usually found. Some tumors show gross local invasion and recurrence, which may indeed kill the patient, but this does not necessarily associate with metastatic potential. Here, we report a case of vertebral metastatic paraganglioma that occurred 19 months after the patient had undergone partial cystectomy for urinary bladder paraganglioma. We believe this to be a rarely reported bone metastasis of paraganglioma arising originally within the urinary bladder. In this report, we also provide a summary of the general characteristics of this disease, together with progress in diagnosis, treatment, and prognosis.     

子宫颈小细胞癌18例临床病例分析

目的探讨子宫颈小细胞癌(SCCC)的临床特点及预后因素。方法对18例SCCC,回顾性总结分析其临床、病理及随访资料。结果本组18例患者的中位年龄为42.5岁(24～65岁)。7例子宫颈伴有其它类型的癌。16例患者行子宫颈癌根治术,6例患者保留一侧卵巢,未见卵巢部位复发。9例有盆腔淋巴结转移,7例治疗后发生远处转移。18例患者中位生存期22个月,总的2年和5年生存率分别为44.4和22.2。16例手术患者9例接受过放疗和7例未接受放疗患者的复发率分别为44.4(4/9)和28.6(2/7)(P=0.039),5年生存率分别为33.3(3/9)和14.3(1/7)(P=0.002)。16例患者1例未接受术后化疗,40周后死亡。结论SCCC预后差,易复发和转移。辅助放疗、化疗可能会提高患者的生存率。     

膀胱小细胞神经内分泌癌与鳞癌混合性癌1例并文献复习

目的:通过对1例膀胱小细胞神经内分泌癌与鳞癌的混合性癌患者病例的分析,探讨该病的临床特点、病理学特点、临床诊断、治疗方法及预后。方法:回顾性分析遵义医学院附属医院泌尿外科2017年10月收治的1例术后病理组织学诊断为膀胱小细胞神经内分泌癌与鳞癌混合性癌患者的临床资料并进行国内外文献复习。结果:49岁男患,因“发现肉眼血尿2月余”入院,全麻下行腹腔镜根治性膀胱全切+回肠新膀胱+扩大淋巴结清扫术,术后经病理组织学确诊为膀胱混合性癌（小细胞神经内分泌癌+角化型鳞癌）。结论:膀胱小细胞癌(包括其与移行细胞癌、腺癌和鳞癌等复合癌)临床表现主要以肉眼血尿为主,呈现高度的恶性生物学行为,具有发现晚、进展快、转移早、恶性程度高、预后差等特点,需尽早完善病理学诊断,明确肿瘤的临床分期以达到早期治疗与有效改善其预后的目的。