Mixed large cell neuroendocrine carcinoma with squamous cell carcinoma of the rectum: Report of a rare case and review of the literature

INTRODUCTIONMixed large cell neuroendocrine neoplasms of the rectum are rare and aggressive neoplasms. Survival is poor due to the high rate of lymph node metastases and distant metastases at the time of diagnosis.PRESENTATION OF CASEWe report a case of a 50-year-old male patient with a mixed large cell neuroendocrine carcinoma with squamous cell carcinoma of the rectum located 8 cm from the anal verge, treated with low anterior resection and total mesorectal excision with free surgical margins. There were lymph nodes metastases but no distant metastases at the time of diagnosis. The patient refused to receive adjuvant chemotherapy and died 6 months later due to liver failure as a result of multiple hepatic metastases.DISCUSSIONThere are not known predisposing factors for the development of neuroendocrine rectal carcinoma. A neuroendocrine carcinoma of the rectum is a rare tumor with an incidence of less than 0.1% of all colorectal malignancies. The median survival ranges from 5 to 10.4 months in several studies and there are not sufficient data in bibliography about ideal adjuvant therapy after resection of mixed squamous large cell neuroendocrine carcinoma of the rectum.CONCLUSIONLow anterior resection and total mesorectal excision with free surgical margins in the presence of lymph nodes metastasis is not a sufficient treatment for rectal neuroendocrine carcinoma. More studies should be done in order to determine the ideal adjuvant treatment of these rare and aggressive tumors.     

Three different types of congenital lacrimal sac fistulas

Three different symptoms in 4 patients with congenital lacrimal sac fistulas are presented. The first symptom was epiphora since birth, the second symptom was infection of the lower eyelid, and the third symptom was tearing from the eye in a 76-year-old patient. This patient had nasolacrimal obstruction at the canal level and no symptoms of congenital lacrimal sac fistula. She had undergone excision, including dacryocystorhinostomy. Three of the 4 patients underwent excision of the fistulous tract. The fistula originated from the lacrimal sac in all patients. Symptomatic congenital lacrimal sac fistulas can be treated successfully with excision alone or with excision and dacryocystorhinostomy in cases of nasolacrimal obstruction.     

Reconstruction of maxillary and palatal defects with the free radial forearm flap

We present six patients with maxillary and palate defects that were reconstructed with the radial forearm flap. Four patients had malignant neoplasms involving the maxilla, three with squamous cell carcinoma and the fourth with recurrent basal cell carcinoma. They were treated with excision and immediate reconstruction using a radial forearm free flap. The other two patients presented with large fistulae between the maxilla and nasal sinuses, these being sequelae of previous surgical treatment for malignancies. The fistulae were closed with radial forearm free flaps. This method provides primary wound healing, restoration of palatal function, preservation of facial contour, and a minimal morbidity while obviating the need for palatal prosthesis. In the six cases, the oral cavity has been completely separated from the paranasal sinus and nasal cavity, and all patients demonstrated satisfactory deglutition and intelligible speech.     

Primary large cell neuroendocrine carcinoma of the urinary bladder

Large cell neuroendocrine carcinoma (LCNEC) of the urinary bladder is rare. It is a type of neuroendocrine carcinoma morphologically distinct from small cell carcinoma. We report here a case of primary LCNEC of the urinary bladder. We observed a very large invasive tumor, which was not able to be detected three months previously by cystoscopy or computed tomography. The tumor cells morphologically and immunohistochemically resembled that of pulmonary LCNEC. With prompt cystoprostatectomy and chemotherapy, the patient is free of disease 16 months after diagnosis. Although LCNEC is usually very aggressive, it may be controlled by early diagnosis and treatment.     

Synchronous and multiple transitional cell carcinoma of the bladder and urachal cyst

Abstract:   Incomplete involution of the allantoic duct can result in different pathological forms of urachus which can give rise to inflammation or late malignant changes. Among urachal tumors, adenocarcinoma is most frequent, although other histological types can also be found. The synchronous presentation of a urachal transitional cell tumor, along with recurrent superficial bladder tumors has not been reported previously. We are reporting a 49-year-old male patient in whom transitional cell carcinoma of a urachal cyst was found with recurrent, multiple bladder tumors. The diagnosis of urachal cyst tumor was established according to ultrasonography and computed tomography. Most of the bladder tumors were resected transurethrally while open surgical excision of the urachal cyst with en bloc resection of the bladder dome was performed. Recurrent bladder tumors were afterwards treated with Bacillus Calmette Guerin (BCG) instillations. A year after surgery the patient has no signs of local recurrence or distant metastases of transitional cell carcinoma.     

Pseudonasolacrimal duct obstruction caused by nasal allergy

While mechanical obstruction of the nasolacrimal duct is the most common cause of acquired unilateral epiphora, it is not the only one. Of the 94 patients evaluated with this symptom, 22 cases (23%) were caused by primary nasal changes in the form of nasal allergy. Many of the characteristic findings of mechanical obstruction were present. Several patients had previous nasolacrimal duct surgery, which failed to eliminate the epiphora. Nasal allergy was not initially considered either by previous examiners or the patients themselves, because of the absence of typical allergic symptoms. All of these patients, however, obtained relief from their unilateral tearing with treatment directed toward the nasal pathology only. Oral sympathomimetics and antihistamines were the most effective. Two patients improved with primary nasal surgery.     

Clinical presentation of epithelioid angiomyolipoma

OBJECTIVE: Epithelioid angiomyolipomas (AML) of the kidney are malignant tumors with aggressive clinical behavior. METHODS: We reviewed cases of epithelioid AML recently diagnosed at our institution to highlight the spectrum of clinical presentations. RESULTS: In all cases, renal lesions seen on computed tomography were suspicious for renal cell carcinoma (RCC). Histologically, these tumors can resemble RCC. The diagnosis of epithelioid AML was established by positive staining for melanoma and smooth muscle cell markers, and presence of perivascular epithelioid cells. One patient presented with a renal tumor extending into the inferior vena cava to the level of the hepatic veins. Two patients developed recurrent, metastatic disease following nephrectomy. One patient with tuberous sclerosis and multiple, bilateral AML developed an enhancing renal tumor that did not contain any fat densities. A partial nephrectomy was performed and pathology revealed epithelioid AML adjacent to conventional AML. CONCLUSIONS: These tumors are distinguished from RCC by positive immunostaining for melanoma markers and smooth muscle cell markers. They resemble conventional RCC on imaging. Epithelioid AML may be locally aggressive and metastasize.     

Basal cell carcinoma mimicking a chronic unstable scar in an adolescent patient with Kindler syndrome

We present the case of Kindler syndrome in a 17-year-old man. After excision, histomorphological analysis, and wound closure of a chronic unstable scar in the right popliteal region, a basal cell carcinoma was diagnosed accidentally. A malignancy was not suspected, although the patient already had biopsies, multiple trials of debridement, and skin grafting earlier elsewhere. While an initial attempt to close the defect with a microsurgical flap was abandoned, due to microvascular irregularities, a wound closure was achieved with a free skin graft. The postoperative histomorphologic analysis revealed the surprising diagnosis of a basal cell carcinoma, fortunately with free margins. At follow-up 1 year later, the grafted area was stable, and there was no recurrent disease. A preoperative histomorphologic analysis of the complete excised chronic wound, especially in known skin disorders—regardless of young age—is mandatory. Furthermore, the presented case showed that an abnormal deposition of collagen, and enhanced coprostasis with migration of granulocytes, destroys the normal consistency of small vessels and presents a limitation for microsurgical procedures in these patients.     

Composite Skin Grafts for Basal Cell Carcinoma Defects of the Nose

Basal cell carcinoma is the most frequent cutaneous cancer of the nose and is characterized by its local spreading and exceptionally rare tendency to metastasize. Since a significant advantage has been seen in surgery compared to other treatments, surgical excision ensuring the highest chance of cure is frequently employed. Excision defects of the nose may be covered with either local flap or a full-thickness skin graft. In resurfacing such defects following excision of basal cell carcinomas, we favor the technique of composite-skin grafting which involves the harvesting of composite-skin graft including the epidermis, dermis and superficial layers of subcutaneous tissue to obtain the required thickness in the recipient site. This technique was used for defects remaining after the excision of basal cell carcinomas in a series of 15 patients. The areas involved were lateral nasal region (5 cases), nasal tip (4 cases), dorsum (3 cases), alar lobule (2 cases), and soft triangle (1 case). The mean follow-up was 14.2 months. The color, texture and thickness of the composite-skin graft harvested from the preauricular site and the neck compare favorably with the skin of the nose region. Satisfactory results, both clinically and in patient appreciation, have been obtained in both the reconstruction site and the appearance of the donor site in all patients.     

Nasal Eosinophilic Angiocentric Fibrosis with Orbital Extension

Eosinophilic angiocentric fibrosis (EAF) is an extremely rare, chronic, benign, idiopathic disorder that mostly affects the upper respiratory tract, particularly the nasal cavity, and features progressive submucosal perivascular fibrosis. To the best of our knowledge, only seven cases of EAF with orbital involvement have been reported. We report a case of sinonasal EAF with orbital extension that presented with left nasolacrimal duct obstruction. A 35-year-old man presented with left epiphora, proptosis, anterolateral globe displacement and nasal obstruction. Endoscopic sinus examination showed a firm, gritty, creamy, yellow, fibrous, adherent mass of maxillary sinus. Diagnosis was established with histopathological examination of excisional biopsy of the lesion. Although EAF is very rare, it should be considered in the differential diagnosis of lesions of upper airway tract, particularly the nasal cavity. Biopsy is necessary for diagnosis and treatment planning. Resecting of the involved tissues completely is essential for prevention of recurrence.     

Metastasis from Renal Cell Carcinoma Presenting as Skeletal Muscle Mass: a Case Report

Renal cell carcinoma can metastasize to virtually any site. Skeletal muscle metastasis is not common. The correct diagnosis of metastatic renal cell carcinoma to skeletal muscle is difficult in comparison with soft-tissue metastasis diagnosis.

We report the case of a 58-year-old man with skeletal muscle metastasis from a clear-type renal cell carcinoma 5 years after total nephrectomy. The tumour was located in the proximal left tight at the level of the great adductor muscle. Clinical work-up included both 18 fluorodeoxyglucose positron emission tomography combined with non-contrast computed tomography and magnetic resonance imaging. The mass was widely excised and was confirmed to be a metastasis from renal cell carcinoma.

Maintaining a high degree of suspicion of metastatic renal cell carcinoma is required for patients with a history of renal cell carcinoma. Positron emission tomography, combined with computed tomography, appears to be an effective surveillance tool. Magnetic resonance imaging is helpful in the differential diagnosis from primary soft-tissue tumours.     

Solitary sebaceous nevus of Jadassohn complicated by squamous cell carcinoma and basal cell carcinoma

BACKGROUND: Sebaceous nevus is a benign congenital epidermal nevus. Its association with basal cell carcinoma is well known. METHOD: This is a case report of sebaceous carcinoma complicated by both basal cell carcinoma and squamous cell carcinoma. RESULTS: The behavior of this tumor is very aggressive, resulting in poor prognosis. CONCLUSIONS: All sebaceous nevi should be excised early.     

Squamous cell carcinoma complicating a chronic lesion of hidradenitis suppurativa: a case report and review of the literature

Squamous cell carcinoma (SCC) arising from chronic hidradenitis suppurativa (HS) is rare; however, the morbidity associated with this presentation is high and management has not been standardised or optimised. We present a case of HS of the perineum and buttocks complicated by SCC, requiring multiple extensive surgical resections. Adjuvant radiotherapy was withheld initially because of concern for poor healing of the surgical wound but was eventually initiated after a second recurrence was identified. The patient ultimately expired 4 years after the initial diagnosis of SCC. We also review 80 cases of SCC complicating HS found in the English literature. Case reports and mechanistic studies suggest the possibility that human papilloma virus and smoking may be risk factors associated with SCC in HS. Despite the majority of SCC cases being well‐differentiated tumours in HS, the highly aggressive nature of SCC in HS and its high likelihood for rapid progression, recurrence, metastasis and high mortality suggests the need to advocate for aggressive treatment. We recommend an aggressive approach to management at the time of SCC diagnosis in HS, which includes appropriate imaging to establish the extent of the tumour, large and deep surgical excision, sentinel lymph node evaluation, consultation with radiation oncology for potential adjuvant radiation therapy and close surveillance.     

Metastatic renal cell carcinoma to the nose and ethmoid sinus

We report a rare case of metastatic renal cell carcinoma involving the nose and ethmoid sinus, 17 years after initial nephrectomy and diagnosis of Stage T1N0 clear cell renal cell carcinoma. The patient complained of nasal obstruction and epistaxis. Intensity-modulated radiotherapy (IMRT) was delivered using a higher-than-conventional daily fraction size concurrently with interferon and thalidomide. The patient achieved a complete response both clinically and by magnetic resonance imaging, without any side effects. Late and rare sites of recurrences and treatment options are discussed.     

Extensive basal cell carcinoma on the right thoracic wall and its distant metastases

Basal cell carcinoma is the most common form of skin cancer, and it very rarely metastasizes. An aggressive case of basal cell carcinoma metastasizing to skin, lungs, posterior chest wall, liver and lymph nodes is presented here.     

局部皮瓣治疗面部基底细胞癌疗效观察

目的:观察局部皮瓣治疗面部基底细胞癌的疗效。方法:47例面部基底细胞癌患者,应用局部皮瓣的方法进行修复,术中做冰冻切片决定手术切缘。结果:所有病例均一期修复,皮瓣存活良好,颜面部外观满意。1例面部其他部位再发基底细胞癌,再次行手术切除,未见复发。结论:面部基底细胞癌切除术后,采用术中冰冻切片快速病理验证的方式可以保证肿瘤的完全切除,应用局部皮瓣修复术后缺损,简便、高效,效果满意。     

Basal cell carcinoma with lung metastasis

A forty-seven year-old man had a small ulceration on his left cheek. The ulcer had a repeated crust formation following removal, for ten years. The patient found a hard tumor in the deep site of the ulcer and the tumor was resected. The pathological diagnosis was basal cell carcinoma. The metastases to the regional lymph nodes developed one year later and these lymph nodes were resected. The histological picture was the same as seen in the primary tumor. Lung metastasis occurred three years later. The tumor was resected and the pathological diagnosis was basal cell carcinoma. Multiple lung metastases developed one year later and massive hemoptysis led to a sudden death two years after the thoracotomy. Thirty-seven cases of basal cell carcinoma with lung metastases were reported in the literature. This case is the thirty-eighth case and the third occurrence in a Japanese.     

Advances in medical imaging for the diagnosis and management of common genitourinary cancers

Medical imaging of the 3 most common genitourinary (GU) cancers—prostate adenocarcinoma, renal cell carcinoma, and urothelial carcinoma of the bladder—has evolved significantly during the last decades. The most commonly used imaging modalities for the diagnosis, staging, and follow-up of GU cancers are computed tomography, magnetic resonance imaging (MRI), and positron emission tomography (PET). Multiplanar multidetector computed tomography and multiparametric MRI with diffusion-weighted imaging are the main imaging modalities for renal cell carcinoma and urothelial carcinoma, and although multiparametric MRI is rapidly becoming the main imaging tool in the evaluation of prostate adenocarcinoma, biopsy is still required for diagnosis. Functional and molecular imaging using 18-fluorodeoxyglucose-PET and sodium fluoride-PET are essential for the diagnosis, and especially follow-up, of metastatic GU tumors. This review provides an overview of the latest advances in the imaging of these 3 major GU cancers.     

Primary Basaloid Carcinoma of the Nipple with Associated Squamous Cell Carcinoma in Situ

Abstract:  Basaloid carcinomas have been documented in various anatomic locations. We describe a primary invasive adenocarcinoma of the nipple with extensive basaloid features that was also associated with squamous cell carcinoma (SCC) in situ and an aggressive behavior. A 69-year-old woman without a history of breast neoplasia presented with right nipple pain. Biopsy of the nipple revealed SCC in situ. One year later, she returned with nipple ulceration. An excisional specimen showed a 1.7 cm nodule composed of invasive sheets and ribbons of basaloid cells with numerous mitoses, extensive tumor necrosis and evidence of glandular differentiation. SCC in situ was present in the overlying epidermis. The differential diagnosis included a primary basaloid adenocarcinoma of the nipple, basal cell carcinoma of the nipple, neuroendocrine carcinoma, melanoma, basaloid variant of adenoid cystic carcinoma and metastatic disease. Immunohistochemical profile of this tumor supported a primary basaloid adenocarcinoma of the nipple. Although the initial sentinel lymph node biopsy was negative, within a year of diagnosis, the patient developed ipsilateral axillary node and pulmonary metastases. To the best of our knowledge, this is the first case of basaloid carcinoma to be documented in this anatomic site.