Lemierre's syndrome: a potentially fatal complication that may require vascular surgical intervention

Septic phlebitis of the internal jugular vein, Lemierre's syndrome, is extremely rare. However, Lemierre's syndrome may cause septic pulmonary emboli or result in fatal systemic sepsis, or both, if a timely diagnosis and appropriate treatment are not provided. We present a case of Lemierre's syndrome that occurred in an otherwise healthy young man. In this case, progression to a moribund state was rapid, and surgical intervention proved lifesaving.     

Chondromyxoid fibroma of the proximal phalanx. A tumour that may be confused with chondrosarcoma

Chondromyxoid fibroma is a rare benign tumour of chondral origin. The diagnosis is difficult, but as Jaffe emphasized, "its recognition is of some importance in that pathologically it may be mistaken for sarcoma and, as such, treated more radically than is necessary" (Jaffe, 1948). Salzer (1965) found only 136 cases in the world literature since 1948 when Jaffe defined the criteria for their histological diagnosis. Only six cases of chondromyxoid fibroma have been reported involving the hand (Frank, Rockwood, 1969; Zatyepin, Klimova, Lipkin, Perety, 1979).     

Experience of three years with intra-operative radiation therapy using the Lyon intra-operative device.

Since November 1985, we have performed 54 Intra-Operative Radiation Therapy (IORT) interventions, essentially in gastric cancer (20 patients) and in pancreatic cancer (22 patients). Mortality, morbidity, and average of survival rates were compared with a non-randomized control group: mortality and morbidity rates were similar in the two groups, with or without IORT. The follow-up period was too short for any valid conclusions about IORT in gastric cancer to be reached. However, in the case of unresectable pancreatic cancer, a significant difference was observed in survival rates when patients were treated by surgery alone or surgery and IORT (4.7 months), and when they were treated by surgery, IORT and external postoperative radiotherapy (8.9 months) (p less than 0.05). The study also examined the relief of abdominal and back pain in patients with unresectable pancreatic cancer: in our experience, survival was longer and more comfortable for patients treated with surgery and IORT. In conclusion, it appears that today IORT is without doubt a good palliative treatment for unresectable pancreatic cancer, but more experience is needed before a conclusion can be reached regarding resectable pancreatic cancer and gastric cancer.     

Chronic spinal arachnoiditis. A postoperative syndrome that may signal its onset

Chronic lumbar radiculopathy following spinal surgery is reported, in which 7 of 25 patients reviewed developed a postoperative syndrome immediately after their original surgery. Later, sometimes years later, all 7 patients developed severe chronic spinal arachnoiditis. This syndrome was characterized by transient violent spasms in the legs, muscle cramps, increased radicular pain, and often fever and chills. The recognition of this syndrome and a proposed method of treatment is discussed.     

Complications of prematurity that may require surgical intervention

Many complications related to prematurity may require surgical intervention. Between July 1981 and July 1987, treatment of patent ductus arteriosus (PDA) (228 patients), necrotizing enterocolitis (NEC) (49 patients), and complications of high-pressure ventilation (eight patients) was reviewed. A PDA was ligated in 136 patients, with one death and one complication. Ninety-two patients had treatment with indomethacin, with 35 failures. A PDA was associated with NEC in 37 of the 49 patients, with a 73% mortality when they occurred within 72 hours of each other. Two patients died following pulmonary resection for lung cysts. The two patients with pneumoperitoneum and pneumopericardium were successfully treated with tube drainage. A PDA ligation was successful, with low mortality and morbidity. Treatment with indomethacin was unsuccessful in 38% of patients. There is a high mortality when NEC and PDA occur within 72 hours of each other.     

Stromal endometriosis of the uterine cervix. A variant of endometriosis that may simulate a sarcoma

Six cases of endometriosis of the uterine cervix characterized by the exclusive or almost exclusive presence of endometriotic stroma are reported. These cases of "stromal endometriosis" were encountered in women 29 to 64 (mean, 43) years of age. Three of them presented with abnormal bleeding, one presented with weight loss and abdominal swelling due to an ovarian clear cell adenocarcinoma, and two were asymptomatic. None of the patients had a history of pelvic endometriosis. In three cases, red lesions of the ectocervical mucosa were recognized on pelvic examination. Histological examination of the lesions showed well circumscribed foci within the superficial stroma of the cervix that were composed of an admixture of closely packed cells resembling endometrial stromal cells, small blood vessels, and extravasated erythrocytes. Endometrial-type glands were absent in the initial sections in all of the cases, but rare glands were found in deeper sections in one case. Apart from the absence or paucity of endometrial glands, the clinical and pathological features of the lesions were similar to those of previously described cases of superficial endometriosis of the cervix. Because of the pure or almost pure stromal composition of the lesion, however, it can be confused histologically with benign and malignant neoplasms, particularly low-grade endometrial stromal sarcoma and Kaposi's sarcoma.     

A complication of the buried suture

A buried suture used in the repair of the central slip of a digital extensor resulted in an erosive lesion of the proximal interphalangeal joint of the long finger. In a review of the literature I can find no other reports describing this unusual complication.     

Angiolymphoid hyperplasia with eosinophilia: A disease that may be confused with malignancy

Twelve new cases of an unusual, benign vasoproliferative and inflammatory disorder of unknown etiology, angiolymphoid hyperplasia with eosinophilia (ALHE), are described and contrasted clinically and pathologically with those appearing in the literature. Only recently recognized in the United States, the disease is of singular importance because the vascular component may be confused histologically with angiosarcoma, thereby resulting in unwarranted aggressive therapeutic measures. ALHE characteristically affects adults and presents in the head and neck region as either solitary or multiple cutaneous tumors. The lesions are pruritic, frequently bleed after minor trauma, and may be associated with peripheral eosinophilia and regional lymphadenopathy. On rare occasions, the disease may masquerade as a salivary-gland tumor, cause stenosis of the external auditory canal, or present as an osseous lesion of the skull. Extrafacial tumors are uncommon. Excision is the most frequent form of therapy; however, local irradiation, corticosteroids, electrodessication with curettage, and chemotherapy have also had varying degrees of success.     

Oxalate in renal stone disease: the terminal metabolite that just won't go away

The incidence of calcium oxalate nephrolithiasis in the US has been increasing throughout the past three decades. Biopsy studies show that both calcium oxalate nephrolithiasis and nephrocalcinosis probably occur by different mechanisms in different subsets of patients. Before more-effective medical therapies can be developed for these conditions, we must understand the mechanisms governing the transport and excretion of oxalate and the interactions of the ion in general and renal physiology. Blood oxalate derives from diet, degradation of ascorbate, and production by the liver and erythrocytes. In mammals, oxalate is a terminal metabolite that must be excreted or sequestered. The kidneys are the primary route of excretion and the site of oxalate's only known function. Oxalate stimulates the uptake of chloride, water, and sodium by the proximal tubule through the exchange of oxalate for sulfate or chloride via the solute carrier SLC26A6. Fecal excretion of oxalate is stimulated by hyperoxalemia in rodents, but no similar phenomenon has been observed in humans. Studies in which rats were treated with (14)C-oxalate have shown that less than 2% of a chronic oxalate load accumulates in the internal organs, plasma, and skeleton. These studies have also demonstrated that there is interindividual variability in the accumulation of oxalate, especially by the kidney. This Review summarizes the transport and function of oxalate in mammalian physiology and the ion's potential roles in nephrolithiasis and nephrocalcinosis.     

Treatment of ampullary villous adenomas that may harbor carcinoma

Villous adenoma of the ampulla of Vater is a rare tumor. It is a deceptive tumor because it is a premalignant lesion and biopsies of the lesion are false negative in 25% to 56% of patients. The primary focus of this report is 23 of 30 patients with villous adenoma of the ampulla of Vater who underwent Whipple operations. Paraffin blocks from 22 patients were available. In eight patients, blocks of the biopsies and the corresponding resected specimens were available. Immunohistochemical studies using antibodies to p53 and Ki-67 were performed to determine whether accumulation of these antibodies in the biopsy specimens would identify false negative biopsies. There was one operative death. The 2-, 5-, and 10-year survival rates for the 22 patients surviving a Whipple operation were 74%, 57%, and 35%, respectively. Three patients died of cancer. The mean p53 expression index was increased in adenomas to 88 (P = 0.001) and in carcinomas to 114 (P = 0.01), compared with 12.6 for normal ampullary epithelium adjacent to tumor. Significant differences in the Ki-67 proliferation index were noted between normal adjacent epithelium (13%), adenoma (34%, P = 0.0002), and carcinoma (53%, P = 0.034), as well as between adenomatous epithelium and carcinoma (34% vs. 53%, P = .012). Villous ampullary adenocarcinoma was present in 65% of patients with villous adenoma (87% if patients with carcinoma in situ in resected specimens are included). Because of the high false negative rate of ampullary biopsies, and the inability to accurately stage these lesions, we recommend pancreaticoduodenectomy in most patients. Studies with p53 and Ki-67 markers suggest that they may be helpful in the recognition of ampullary villous cancer not identified on routine biopsies.