Sclerosing sweat duct (syringomatous) carcinoma

This report describes 20 sclerosing adnexal carcinomas of the skin that recapitulate the sweat duct and share certain microscopic features with benign syringoma. Clinically, the neoplasms were slow-growing, indurated nodules or plaques. Sixteen were located on the face, including five on the upper lip. Three were situated in the axilla, and one involved the buttock. Twelve of the tumors (group 1) were composed primarily of cords and nests of whorled squamous cells with clear cell change and variable keratinization. Lumens consistent with ductal differentiation were few and isolated in some of these lesions, but others had more numerous and complex ducts. The remaining eight neoplasms (group 2) contained large numbers of small, uniform ducts, and exhibited little keratinization. Each neoplasm in the series infiltrated subcutaneous tissue or deeper tissues and was accompanied by sclerotic, fibrous stroma. Sixteen of the lesions (80%) exhibited perineural growth. One tumor involved an underlying lymph node, probably by direct extension. Cytologic atypicality was mild or lacking, and mitotic figures were sparse. Seven of 15 tumors (47%) with follow-up recurred, in some instances many years after primary excision. Tumor-free margins in the initial excision specimen appeared to be a favorable prognostic factor. The most aggressive lesion in the series showed increased cytologic atypicality in recurrences.     

Infiltrating syringomatous adenoma of the nipple.

Infiltrating syringomatous adenoma of the nipple is a distinct, benign clinical entity. It is similar histologically to a syringoma, a benign tumor originating in the ducts of the dermal sweat glands. When located in the nipple, this lesion has been mistaken for nipple duct adenoma or tubular carcinoma. Infiltrating syringomatous adenoma of the nipple is locally infiltrating but does not metastasize. Appropriate local management depends on an accurate diagnosis. Following is a case report, review of the literature, and therapeutic options for infiltrating syringomatous adenoma of the nipple.     

Aggressive surgery for carcinoma of the gallbladder

Forty patients with gallbladder cancer were admitted to our institution in a 9-year period. For two patients with Nevin's stage I carcinoma who had undergone cholecystectomy, resection of the lower portion of the fourth and fifth segments of the liver and extrahepatic bile duct with dissection of lymph nodes was carried out as a second-stage operation. Thirteen patients with stage V carcinoma underwent extensive aggressive operations. Operative procedures comprised various types of liver resection with cholecystectomy and extrahepatic bile duct resection and wide lymph node dissection in all cases, portal vein reconstruction in 3, pancreatoduodenectomy in 3, partial colectomy in 3, and right nephrectomy in 1. The operative and in-hospital mortality rates were 0%. Two patients with stage I carcinoma are both doing well. Two patients with stage V carcinoma who underwent an extended operation are working without recurrence 7 years 8 months and 8 years 5 months after surgery. From our experiences we believe that long-term survival may be achieved by aggressive surgery if it is suitably indicated.     

Infiltrating syringomatous adenoma of nipple

Infiltrating syringomatous adenoma of the nipple is a rare, benign lesion with distinct clinical and histological features. Origin from ducts of dermal sweat glands has been postulated. Important differential diagnosis include nipple adenoma, tubular carcinoma and adenosquamous carcinoma. Appropriate local management includes accurate diagnosis and complete excision to avoid local recurrences. This report describes the condition in a 39-year-old parous lady.     

Aggressive resection for advanced pancreatic carcinoma

An aggressive pancreatectomy was performed on a 53 year old Japanese man with advanced cancer of the pancreas. The tumor originated from the body of the pancreas and invaded the stomach, duodenum, left kidney, transverse colon and common hepatic artery. An unexpected cancer was also found in the head of the pancreas during the operation. Therefore, total pancreatectomy, total gastrectomy, left adrenonephrectomy, resection of the left transverse colon and dissection of the regional lymph nodes were performed. Resection of the common hepatic artery was also performed, followed by an end-to-end anastomosis between the common hepatic artery and celiac trunk. The postoperative course was uneventful and the patient was doing well until nine months after the operation when multiple metastatic lesions were noted in the liver. He died 391 days after the operation from hepatic failure.     

Aggressive resection of the airway invaded by thyroid carcinoma

BACKGROUND: The aim of this study was to investigate the hypothesis that outcome following concomitant airway resection is superior to that after shaving of the tumour in patients with airway invasion of thyroid carcinoma. METHODS: The records of 34 patients with thyroid cancer with airway invasion were reviewed retrospectively. In addition to total thyroidectomy, airway resection was performed in 18 patients (group 1), whereas the tumour was shaved away from the airway in the other 16 patients (group 2). 131I was used as postoperative adjuvant therapy in all patients. Metastasis and recurrence of the primary lesion were determined by 131I whole-body scans, serum thyroglobulin levels, and computed tomography or ultrasonography of the neck. RESULTS: In group 1, two anastomotic dehiscences resulted in one death. Patients in group 2 had a higher rate of local recurrence (relative risk 8.0, P = 0.013) and earlier recurrence (mean(s.e.m.) 2.6(0.8) versus 7.0(1.1) years; P = 0.026) than those in group 1. Median survival was 5.8 and 4.3 years in the 18 patients of group 1 and 16 patients of group 2 (P = 0.259), and the respective 5-year survival rates were 88 and 84 per cent (P = 0.783). CONCLUSION: Aggressive airway resection can minimize local recurrence of thyroid carcinoma with airway invasion.     

Aggressive surgical resection for carcinoma of gallbladder

BACKGROUND: The aim of the present study was to review the long-term result of patients with carcinoma of gallbladder with adequate investigations and aggressive surgical resection. METHODS: A retrospective study was carried out on patients with carcinoma of gallbladder treated at the Division of Hepatopancreatobiliary Surgery, the Chinese University of Hong Kong from January 1997 to December 2002. RESULTS: Of the 47 patients with carcinoma of gallbladder, 28 patients were treated symptomatically because they were found to have metastatic disease (n = 22), unresectable disease (n = 2) or poor associated medical disease (n = 4). One patient had carcinoma in-situ after laparoscopic cholecystectomy. No further treatment was considered necessary. The remaining 18 patients (38.3%) came to laparotomy after preoperative investigations with ultrasonography, computed tomography, endoscopic or percutaneous cholangiography, and visceral angiography. Laparoscopy and laparoscopic ultrasound were used in the later part of the study. Radical cholecystectomy was carried out in 14 patients (77.8%). The in-hospital mortality rate was 5.3% and the surgical morbidity was 31.6%. The remaining four patients underwent palliative cholecystectomy because of the extent of the disease. The overall 5-year survival for the 47 patients was 19.1%, for the 18 patients who came to laparotomy was 44.4%, and for the 14 patients who underwent resection with curative intent was 57.1%. CONCLUSIONS: With adequate preoperative investigations, and the use of laparoscopy and laparoscopic ultrasound, radical resection was possible in the majority of patients who came to laparotomy for carcinoma of gallbladder. Aggressive radical resection gave good results.     

Aggressive surgical management of fibrolamellar hepatocellular carcinoma

Fibrolamellar hepatocellular carcinoma (FLHC) is recognized as a distinct clinicopathologic variant of hepatocellular carcinoma. Ten consecutive patients with FLHC undergoing operative management at our institution were reviewed. At the initial presentation seven patients had stage II disease (pT₂N₀M₀), whereas three patients were in stage III (pT₂N₀M₀ or pT₃N₀M₀). Initial procedures included formal right or left hepatectomy in four patients, right or left trisegmentectomy in two patients, left lateral segmentectomy or nonanatomic resection in three patients, and in one patient considered for liver transplantation, only exploration with biopsy of positive nodes was performed. Four stage II patients required a second procedure for resection of recurrent disease from 8 months to 6 years after the initial resection and one patient required a third procedure after 13 years. Reoperations included hepatic re-resection, resection of extrahepatic disease, and liver transplantation. Overall 5- and 10-year Kaplan-Meier survival was 70%. There were no deaths among stage II patients (follow-up 96 to 180 months). All stage III patients (i.e., lymph node involvement, vascular invasion, or multiple tumors) died within 5 years. Patients with stage II disease had better survival than patients with stage III disease (P=0.011, log-rank test). Aggressive treatment of FLHC including reoperation and liver transplantation is justified, especially in patients with stage II disease.     

Aggressive renal cell carcinoma in a 27-year-old kidney transplant.

Sir, The late development of a primary tumour in an allograft kidneyis a rare event: the longest reported interval period, to ourknowledge, is 21 years. In the case described below, we presentthe finding of an aggressive renal cell carcinoma in a transplantedkidney after 27 years. A 29-year-old     

Perineural invasion of the orbit by squamous cell carcinoma

Two cases of orbital extension by squamous cell carcinoma along the supraorbital nerve are reported. Important clinical aspects of these cases include previous incomplete removal of squamous cell carcinoma of the forehead and altered sensation in the dermatome of the supraorbital nerve. Axial spread of carcinoma along periorbital nerves is an important avenue of local metastasis and should be suspected in all cases of periorbital squamous cell carcinoma.     

Aggressive squamous cell carcinoma in Kindler syndrome

A 57-year-old Hispanic man with a personal and family history of bullae and photosensitivity presented with a fungating, ulcerated squamous cell carcinoma on his left hand (Figure 1). Physical examination showed conjunctival injection, ectropion, symblepharon, urethral stricture, loss of teeth, short stature, and nail dystrophy. There was reticulated erythema, atrophy, hyperpigmentation and hypopigmentation, and telangiectasia of sun-exposed skin of the face, neck, and hands consistent with poikiloderma (Figure 2). In addition, there was foreshortening of the left thumb and sclerodermoid changes of his hands (Figure 3). Radiation therapy was applied to shrink the tumor before a local excision was performed. However, a local recurrence followed and axillary lymph nodes became clinically palpable, necessitating amputation and lymph node dissection. Extensive histologic evaluation of the specimen obtained following left arm amputation and lymph node dissection showed moderate-to-poorly differentiated deeply invasive squamous cell carcinoma. Two of 3 axillary lymph nodes were positive for metastatic carcinoma. A random biopsy of the trunk showed epidermal atrophy, telangiectasia, a perivascular lymphocytic infiltration, and pigment-laden macrophages consistent with poikiloderma. Electron microscopy illustrated extensive reduplication of the basement membrane, with loops, curls, and free extensions of the basal lamina in the superficial dermis; reduced numbers of hemidesmosomes and anchoring fibrils; and a basement membrane focally devoid of basal cells (Figure 4). On the basis of the clinical features and the characteristic basement zone changes, a diagnosis of Kindler syndrome was made.     

Osteomas of the orbit.

Clinical and histologic features of osteomas are reviewed. Techniques are described for the evaluation of osteomas, including the use of coronal and axial plane computed tomography. A series of 14 patients evaluated at the Massachusetts Eye and Ear Infirmary over a five-year period is presented. Two of these patients are discussed in detail. Two surgical approaches to osteomas of the frontal sinuses are described. A brow incision is recommended for tumors primarily within the orbit. A coronal incision and osteoplastic operation is recommended for tumors primarily within the sinuses. In one patient, unusually rapid tumor growth is documented by x-rays taken over a three-year period.     

Aggressive surgery for renal cell carcinoma with ena cava tumor thrombus.

Herein is presented what we believe to be the eighth case reported of renal cell carcinoma with caval tumor thrombus treated by radical excision with vena caval resection. A review of the literature reveals that although postoperative morbidity is minimal, only 1 patient survived more than two years. All patients with metastases at time of operation died within one year. It is our conclusion that the length and quality of survival anticipated must be carefully weighed before performing this major surgical procedure.     

Aggressive fibromatosis.

Ten patients with aggressive fibromatosis of the extremities were prospectively followed for 2-6 years. Results of treatment methods were compared. Five patients underwent three-dimensional imaging with and without intravenous contrast, and the images were compared with the anatomic extent of the resected lesion. Pathologic specimens and control tissue were tested for the presence of estrogen and progesterone receptors and for expression of the c-sis oncogene and platelet-derived growth factor (PDGF), potent mitogens for fibrocytes. Wider surgical resection resulted in a lower recurrence rate, but current chemotherapeutic agents were not effective in eradicating the tumors. Intravenous contrast enhanced the lesions. Two thirds of the tumors tested had estrogen or progesterone receptors. All tumors tested had inappropriate expression of c-sis and PDGF. This inappropriate expression may be responsible for the underlying pathobiology and deregulation of control of growth in aggressive fibromatosis.     

Infiltrating syringomatous adenoma of the nipple. A clinical and pathological study of 11 cases

The clinical and pathologic findings of 11 infiltrating syringomatous adenomas of the nipple (ISA) were studied. All neoplasms were composed of small ducts and solid strands of epithelial cells surrounded by desmoplastic stroma. Ten of the 11 invaded the smooth muscle of the nipple, four extended to underlying breast tissue, and one showed perineural invasion. All lesions had an infiltrative margin, but 10 were treated successfully by local excision, even though five (45%) recurred. None metastasized. ISA must be distinguished from nipple duct adenoma and tubular carcinoma. Its clinical significance lies primarily in its recognition as a distinctive benign neoplasm. In the past, a variety of terms have been used to describe this lesion, whether it occurred in the skin, nipple, or substance of the breast. "Infiltrating syringomatous adenoma" is the preferred term to avoid using "carcinoma" for lesions involving the breast.     

Cavernous hemangioma of the orbit.

The clinical and pathological data of 66 patients with orbital cavernous hemangioma are presented. This tumor occurs in females more frequently than in males, and has its peak incidence in early middle age. Visual disability results from a high degree of relative hyperopia or from optic-nerve compression. Postural or temporal variation is proptosis is not characteristic. Multiple cavernous hemangiomas are rare, but may occur simultaneously or separated by long intervals. In this series, incompletely excised lesions did not cause recurrent proptosis. Relative hyperopia may persist, in spite of complete removal of the tumor. Improved preoperative localization with modern techniques appears to be reducing the morbidity associated with surgical excision of the lesion. A local hemodynamic disturbance may initiate proliferation of vascular channels that undergo progressive ectasia. Growth of the lesion may occur intrinsically by the budding-off of capillary channels from cavernous spaces into the interstitium. Clinical and pathological findings fail to demonstrate any relationship between this lesion and capillary hemangioma of childhood.