The histopathological classification, diagnosis and differential diagnosis of mucinous appendiceal neoplasms, appendiceal adenocarcinomas and pseudomyxoma peritonei.

The vermiform appendix is the primary site of several distinctive benign and malignant neoplasms. Some can produce the clinical syndrome of pseudomyxoma peritonei (PMP). A consensus on their terminology was reached by an international panel of pathologists and clinicians working under the auspices of the Peritoneal Surface Oncology Group International (PSOGI), and this review discusses the application of the PSOGI classification to routine reporting. We discuss diagnosis and differential diagnosis together with implications for patient management, covering low-grade appendiceal mucinous neoplasms, high-grade appendiceal mucinous neoplasms, serrated polyps, adenomas and adenocarcinomas. We do not cover goblet cell tumours or neuroendocrine neoplasms in this paper.

Pleuropulmonary involvement in pseudomyxoma peritonei: morphologic assessment and literature review.

Intrathoracic spread in patients with pseudomyxoma peritonei (PP) is rare. We reviewed 101 patients uniformly treated at our institution for PP of appendiceal origin. In that study, we suggested mucinous carcinoma peritonei (MCP) as the pathologic terminology for all cases of PP. Four patients had pathologically documented pleuropulmonary involvement. We subsequently examined another patient with pleural invasion. Of 5 patients, 3 had low-grade histologic features in the peritoneum; these showed variably proliferative, bland-appearing neoplastic cells arising from low-grade appendiceal mucinous neoplasms. In 2 cases, 1 or more pulmonary parenchymal metastases of low histologic grade developed. The lack of pleural involvement argued against transdiaphragmatic tumor extension. The third patient with low-grade MCP had direct extension through the left diaphragm involving the left pleural and pericardial spaces without pulmonary parenchymal involvement. In the 2 patients with high-grade MCP, right-sided pleural effusions developed. Neither patient had documented injury to the diaphragm. Pleural cytologic examination revealed high-grade adenocarcinoma cells singly, in small clusters, and in large spheres. The smear backgrounds contained wispy mucin. None of the 5 patients developed thoracic lymph nodal metastases. Although rare, mucinous neoplasms from PP may involve the thorax.

Pseudomyxoma peritonei of appendiceal origin: a clinicopathologic analysis of 101 patients uniformly treated at a single institution, with literature review.

Pseudomyxoma peritonei is a clinical term for gelatinous ascites, usually secondary to an appendiceal tumor. The pathologic classification of pseudomyxoma peritonei and its associated appendiceal tumors has been plagued with controversy and confusing terminology. In an effort to clarify this, we reviewed the pathology of 101 patients, all treated at our institution from 1993 to 2005, with pseudomyxoma peritonei of appendiceal origin. All patients were uniformly treated with our standardized protocol. This is the largest pathologic series solely devoted to appendiceal neoplasia with gelatinous ascites. The cases were assigned, according to previously published criteria, to the categories of disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), or PMCA with intermediate (well differentiated) features (PMCA-I), with the exception that any case with a signet-ring cell component was considered as PMCA and not PMCA-I. By histologic category, 58 patients had DPAM, 23 were PMCA, and 20 were PMCA-I.One-year, 3-year, and 5-year survival outcomes were not significantly different between DPAM and PMCA-I. DPAM and PMCA-I also exhibited a roughly equal incidence of parenchymal (beyond the serosa) organ invasion. Survival outcomes were significantly worse for PMCA, compared with PMCA-I and DPAM. After reviewing our data and the literature, mucinous carcinoma peritonei-low grade was applied to the low-grade histology of pseudomyxoma peritonei, including those cases referred to by some as DPAM in the same category as PMCA-I. Cases that are moderately differentiated to poorly differentiated are classified as mucinous carcinoma peritonei-high grade.

Intraperitoneal hemorrhage in a patient with hepatic focal nodular hyperplasia.

Focal nodular hyperplasia (FNH) is a common benign liver tumor that is often confused with hepatic adenoma. Despite the benign course of both lesions, hepatic adenomas are associated with rupture and bleeding, and suggested management often includes prophylactic surgical resection. FNH lesions are thought to not rupture or bleed, and traditional management does not include resection. We report the case of a woman with FNH who presented with intraperitoneal hemorrhage after the rupture of a FNH lesion. Only six previous documented cases of hemoperitoneum associated with FNH have ever been reported. This report reviews all previously recorded cases of FNH lesions that have resulted in intraperitoneal hemorrhage. We suggest that although FNH is a benign lesion and intervention is typically unnecessary, the potential for rupture requiring surgical resection should always be considered.

Resection of pulmonary metastasis from parathyroid carcinoma.

The management of metastasis from parathyroid carcinoma (PC) is an unusual challenge. Systemic therapy has minimal effect on the course of the disease and its associated hypercalcemia. Resection of isolated pulmonary metastases is an attractive option in the setting of recurrent hypercalcemia. We report a case of a patient who underwent resection of multiple functional PC pulmonary metastases almost 20 years after the original neck resection. The patient originally presented in 1982 with what was thought to be hyperparathyroidism secondary to a parathyroid adenoma. Surgical exploration revealed the presence of a well differentiated PC. The patient was transiently normocalcemic, but underwent surgical exploration in 1993 and 1996 for recurrent PC in the neck. The patient again improved, but presented in 2001 with diffuse arthralgia, fatigue, malaise, weight loss, and decreased appetite. She was found to have a serum calcium of 12.7 mg/dL and an intact parathyroid hormone of 1542 pmol/L. Computed tomography identified the presence of two right-sided and two left-sided pulmonary masses. Sternotomy with bilateral pulmonary metastectomy was performed. Functional PC metastasis was confirmed on pathological examination. One year following metastectomy the patient is hypocalcemic, requiring oral calcium and vitamin D supplementation. Functional pulmonary metastasis from PC is a rare cause of hypercalcemia. Complete resection of isolated pulmonary metastases can provide effective palliation with long-term survival.

Bronchoscopic view of pulmonary blastomycosis.

Blastomycosis is a chronic fungal infection often diagnosed in the south-central and midwestern United States. Lungs are primarily involved, with lymphatic or hematogenous spread to other organs. Bronchoscopy is considered when the noninvasive tests fail to establish the diagnosis. We describe bronchoscopic finding of blastomycosis involving the upper and lower respiratory tract. Inhaled steroids may have suppressed cellular immunity, allowing fungus to grow in the submucosa with histology significant for extensive eosinophilic infiltration. To our knowledge, such a detailed pictorial description of pulmonary blastomycosis with a cobblestone appearance of the tracheobronchial mucosa has not been reported in the past.

Congenital visceral myopathy with a predominantly hypertrophic pattern treated by multivisceral transplantation.

Chronic intestinal pseudoobstruction due to visceral myopathy may cause intestinal failure. The anatomical pathology of visceral myopathy is dominated by an atrophic muscularis propria. We present a case that is overwhelmingly dominated by a hypertrophic muscularis propria. The hypertrophic pattern was documented from ages 2 to 28 years. Most descriptions of visceral myopathy focus on the atrophic pattern of injury to the muscularis propria, typified by vacuolar degeneration of myocytes, loss of muscle fibers, and frequently with a honeycomb pattern of fibrosis. Hypertrophic changes can be observed in visceral myopathy, but the hypertrophy typically represents only a minor feature, typically limited to the internal layer of the muscularis propria. In our patient, the observed muscular hypertrophy differed from most descriptions because it (a) was the dominant pattern of injury and (b) involved both layers of muscularis propria rather than just the inner circular layer. The patient had intestinal failure and the complications induced cirrhosis, eventually treated by multivisceral transplantation to correct the intestinal failure and cirrhosis.

Recurrent hepatic lymphangiomatosis after orthotopic liver transplantation.

Hepatic lymphangiomatosis is a rare disease characterized by an abnormal lymphatic proliferation involving the liver alone, liver and spleen, or multiple organs. Hepatic lymphangiomatosis becomes symptomatic secondary to compression or replacement of the normal parenchyma, which can lead to liver failure. Resection and orthotopic liver transplantation (OLT) can be used as treatment for this disease. We herein describe a 42-year-old female who had undergone successful OLT for hepatic lymphangiomatosis with recurrent disease detected 19 yr later in the transplanted liver. This is, to our knowledge, the first described case of recurrent hepatic lymphangiomatosis after OLT. In conclusion, we discuss the clinical, radiologic, pathologic, and immunohistochemical findings and review other reported cases of hepatic lymphangiomatosis that have undergone OLT.

Appendiceal neoplasms with peritoneal dissemination: outcomes after cytoreductive surgery and intraperitoneal hyperthermic chemotherapy.

BACKGROUND: Appendiceal neoplasms frequently present with peritoneal dissemination (PD) and have a clinical course marked by bowel obstruction and subsequent death. Few data have correlated outcome with appendiceal histology after cytoreductive surgery and intraperitoneal hyperthermic chemotherapy (IPHC). We have reviewed our experience with cytoreductive surgery and IPHC for PD from the appendix. METHODS: A total of 110 cases of PD from proven appendiceal neoplasms treated with IPHC were identified from a prospectively managed database. Tumor samples were classified on pathologic review as disseminated peritoneal adenomucinosis (n = 55), peritoneal mucinous carcinomatosis (PMCA) with intermediate features (n = 18), PMCA (n = 29), or high-grade nonmucinous lesions (n = 8). A retrospective review was performed with long-term survival as the primary outcome measure. RESULTS: A total of 116 IPHCs were performed on 110 patients for appendiceal PD between 1993 and 2004. The 1-, 3-, and 5-year survival rates for all cases were 79.9% +/- 4.1%, 59.0% +/- 5.7%, and 53.4% +/- 6.5%, respectively. When stratified by histology, disseminated peritoneal adenomucinosis and intermediate tumors had better 3-year survival rates (77% +/- 7% and 81% +/- 10%) than PMCA and high-grade nonmucinous lesions (35% +/- 10% and 15% +/- 14%; P = .0032 for test of differences between groups). Age at presentation (P = .0134), performance status (P < .0001), time between diagnosis and IPHC (P = .0011), resection status (P = .0044), and length of hyperthermic chemoperfusion (P = .0193) were independently associated with survival. CONCLUSIONS: The data show that long-term survival is anticipated in most patients who are treated with cytoreduction and IPHC for appendiceal PD. The findings presented herein underscore the important prognostic characteristics that predict outcome after IPHC in patients with PD. In all, this work establishes a framework for the consideration of IPHC in future trials for appendiceal PD.