Neonatal lupus erythematosus presenting as a non-bullous histiocytoid neutrophilic dermatosis.

Histopathologic findings in neonatal lupus erythematosus (NLE) are usually congruent with those of subacute cutaneous lupus erythematosus. However, neutrophilic dermatosis-type histopathologic features are being increasingly recognized in the literature including rare cases with variant histiocytoid morphology. We report the case of a 7-week-old male presenting with figurate erythema. His mother was found to have elevated anti-nuclear antibodies and was positive for anti-SSA/Ro, anti-SSB/La antibodies and Ro52 autoantibodies. The infant had a similar serological profile. Skin biopsy showed a histiocytoid interstitial infiltrate with mild lichenoid features, sparse neutrophils and mild leukocytoclasis. The histiocytoid infiltrate showed prominent CD68, CD163, and myeloperoxidase expression. Isolated clusters of CD123+ histiocytes were also present. This case highlights the rare finding of non-bullous neutrophilic dermatosis with histiocytoid change in neonatal lupus. In neonates presenting with figurate erythemas with morphological histiocytic change on biopsy, NLE should be considered as a differential diagnosis and investigated for accordingly.

Rare Mimic of a Myxofibrosarcoma: Cutaneous Myxoid Spindle Cell Squamous Cell Carcinoma as a Complication of Chronic Osteomyelitis.

ABSTRACT: Myxoid spindle cell squamous cell carcinoma is a rare variant of squamous cell carcinoma that can pose diagnostic challenges because of its unusual morphology. In this article, we report the case of a 68-year-old man who presented with a slow-growing, fungating mass on the right tibia at the site of his long-standing draining sinus tract. Biopsy revealed a malignant spindle cell tumor with prominent myxoid stroma and areas containing thin-walled blood vessels with a curvilinear appearance. The immunohistochemical profile indicated that the neoplastic cells were positive for a variety of keratins (MNF116, Cam 5.2, AE1/AE3, 34ßE12, and CK5/6) and transcriptional markers classically expressed in squamous cell carcinomas (p63 and p40). The tumor cells were negative for melanocytic and mesenchymal markers smooth muscle antibody, S100, caldesmon-h, desmin and CD34. Together, the clinical history, histologic appearance, and immunohistochemical panel was diagnostic of a myxoid spindle cell squamous cell carcinoma. The main differential diagnosis was myxofibrosarcoma. In addition to this clinical case, we also outline the current state of knowledge on this rare entity and discuss the importance of recognizing a Marjolin ulcer in this scenario.

Cryptococcosis presenting as upper limb cellulitis and ulceration: a case series.

We report four cases of cryptococcosis presenting as upper limb cellulitis or ulceration, or both. Three of the four patients were on long-term prednisolone therapy at the time of presentation. In each case, the diagnosis of cryptococcosis was established by a biopsy of the skin. Only one of the four patients had conclusive evidence of disseminated disease. Our cases highlight the importance of skin biopsy in immunosuppressed individuals presenting with cellulitis, particularly when the cellulitis occurs in an atypical location and when the clinical condition fails to respond to standard antibacterial therapy.

Direct tumor invasion in colon cancer: correlation with tumor spread and survival.

PURPOSE: This study examined the correlation between depth of local invasion in colon cancer and tumor spread and patient survival. METHODS: A cohort of 796 patients with a complete set of TNM staging information following an elective resection for colon cancer was selected. The rates of lymph node and distant metastasis, tumor differentiation, and extramural venous invasion for different tumor (T) categories were compared. The effects of initial tumor (T) category on overall patient survival were studied. RESULTS: The depth of local tumor invasion correlated strongly with nodal involvement (P = 0.0001), rates of extramural venous invasion (P = 0.0002), poor differentiation (P = 0.0001), and distant metastasis (P = 0.0001). Fifty-seven percent of the patients remained lymph node-negative and distant metastasis-negative irrespective of their depth of tumor invasion had no impact on overall survival (P = 0.49). For patients with lymph node or distant metastasis (43 percent), depth of tumor invasion had significant impact on overall survival (P = 0.001). Thirteen percent of T3N1, 33 percent of T3N2, 40 percent of T4N1, and 68.percent of T4N2 cases had distant metastasis at presentation. CONCLUSION: Two types of colon cancer were observed: locally active and tendency to metastasize. For the latter, overall mortality and the risk of metastasis increased with depth of tumor invasion.

PTEN mutations are common in sporadic microsatellite stable colorectal cancer.

The tumour suppressor gene PTEN, located at chromosome sub-band 10q23.3, encodes a dual-specificity phosphatase that negatively regulates the phosphatidylinositol 3'-kinase (PI3 K)/Akt-dependent cellular survival pathway. PTEN is frequently inactivated in many tumour types including glioblastoma, prostate and endometrial cancers. While initial studies reported that PTEN gene mutations were rare in colorectal cancer, more recent reports have shown an approximate 18% incidence of somatic PTEN mutations in colorectal tumours exhibiting microsatellite instability (MSI+). To verify the role of this gene in colorectal tumorigenesis, we analysed paired normal and tumour DNA from 41 unselected primary sporadic colorectal cancers for PTEN inactivation by mutation and/or allelic loss. We now report PTEN gene mutations in 19.5% (8/41) of tumours and allele loss, including all or part of the PTEN gene, in a further 17% (7/41) of the cases. Both PTEN alleles were affected in over half (9/15) of these cases showing PTEN genetic abnormalities. Using immunohistochemistry, we have further shown that all tumours harbouring PTEN alterations have either reduced or absent PTEN expression and this correlated strongly with later clinical stage of tumour at presentation (P=0.02). In contrast to previous reports, all but one of the tumours with PTEN gene mutations were microsatellite stable (MSI-), suggesting that PTEN is involved in a distinct pathway of colorectal tumorigenesis that is separate from the pathway of mismatch repair deficiency. This work therefore establishes the importance of PTEN in primary sporadic colorectal cancer.

Surgical management of colorectal cancer in south-western Sydney 1997-2001: a prospective series of 1293 unselected cases from six public hospitals.

BACKGROUND: The aim of the present study is to provide local data for the management of colorectal cancers in the south-western Sydney health area from 1997 to 2001. METHODS: The data were collected prospectively. Follow up was conducted in late 2001 and early 2002. Data were cross-validated with hospital and area databases and with data from the New South Wales Registry of Births, Deaths and Marriages. RESULTS: This was an unselected series of 1293 patients from 36 surgeons; 16.5% of patients presented as emergencies. Only 3% presented as a result of bowel cancer screening. Of the 1293 patients, 1270 received an operation. There were 598 elective colonic resections with the mortality rate of 1.2%, reoperation rate of 2.7% and anastomotic leak rate of 0.8%. For the 410 elective rectal resections, the rates were 2.9%, 2.7% and 1.2%, respectively. For the 290 emergency operations, the rates were much worse at 7.7%, 6.6% and 4.8%, respectively. The corrected overall 3-year survival rate was 64%. For Dukes' A, B, C and D, the figures were 94%, 87%, 61% and 7%, respectively. CONCLUSIONS: Colorectal cancer is a major cause of mortality and morbidity in our community. Very few bowel cancers were discovered at the asymptomatic stage. This paper strongly supports community bowel cancer screening and early diagnosis. The local database has provided a rich source of information to benchmark management and outcomes of bowel cancer patients treated in the South Western Sydney Area Health Service. An area-wide computer network with online data input facilities at individual workplaces will improve data integrity and data collection efficiency.

Serous oligocystic adenoma of the pancreas: a clinicopathological and immunohistochemical study of three cases with ultrastructural findings.

AIMS: Serous oligocystic adenoma of the pancreas is an uncommon benign neoplasm and is a recently described entity. To date, there are 19 adult cases of this tumour. We report three additional cases, two with macrocystic and one with unilocular types. We describe their clinicopathological, immunohistochemical and ultrastructural findings and review the world's literature. METHODS: For a 10-year period, we reviewed all benign cystic lesions of the pancreas with emphasis on serous oligocystic adenoma. We characterised serous oligocystic adenoma as an ill-demarcated or encapsulated mass, composed largely or exclusively of macrocysts (cysts measuring 20mm or more) but few in number (oligolocular). Grossly, it may contain only a single cyst (unilocular) of any size with a few satellite cysts observed on histological examination. Special stains and immunohistochemistry as well as electron microscopy were performed on three and two cases of serous oligocystic adenoma, respectively. RESULTS: Between 1990 and 2000, we collected 26 benign cystic lesions of the pancreas, three of which were serous oligocystic adenomas (two with macrocystic and one with unilocular types). Many of the cells lining the cysts showed PAS positivity. There was negative staining for PAS with diastase digestion, Alcian blue and mucicarmine. All cases showed positive staining for CAM5.2, AE1/AE3, EMA and CK7. The proliferation index marker was low. There was negative staining for CK20, insulin, glucagon, somatostatin, synaptophysin, chromogranin A, CEA and p53. Ultrastructural studies on two cases revealed similar findings. The single row of uniform epithelial cells lining the cysts was composed of simple cuboidal to flat cells which rested on a thin basal lamina. Their nuclei were round to ovoid. Glycogen granules were identified in the cytoplasm. Short microvilli emerged from the epithelial apical surface. Adjacent tumour cells were connected by microfilaments. CONCLUSIONS: Serous oligocystic adenomas of the pancreas are uncommon benign tumours. Prior to this study, 19 adults with these lesions were reported in the world's literature. No correct pre-operative diagnosis was carried out on all 22 cases. The 20 patients with follow-up ranging from 2 months to 5 years did not show tumour recurrence or malignant transformation.

Cutaneous sarcoidosis in a patient with ulcerative colitis on infliximab.

The advance of anti-tumour necrosis factor (TNF) therapy had dramatically changed the treatment algorithm of inflammatory bowel disease (IBD). This had significantly improved the quality of life for patients with Crohn's disease (CD) and ulcerative colitis (UC).(1) However, side-effects of anti-TNF treatment were unavoidable with paradoxical inflammation (for example leucocytoclastic vasculitis and psoriasis) being well-known phenomena of anti-TNF therapy.(2) We report a case of infliximab induced cutaneous sarcoidosis in a patient with ulcerative colitis and review the literature.