Congenital Pseudarthrosis of Femur and Sciatic Nerve Palsy with Congenital Constriction Band Syndrome: Report of the First Case in Literature

Congenital constriction band syndrome has varied clinical presentations ranging from small, incomplete skin deep constriction band to in utero amputation. Pseudarthrosis of underlying bone most commonly tibia has been reported by many authors. We report the first case of congenital pseudarthrosis of the femur with congenital constriction band syndrome. Nine-day-old female presented with the constriction band in the left thigh with open pseudarthrosis of the femur. The left femur had gross recurvatum deformity and the posterior apex of the pseudarthrosis was exposed via skin ulceration. She had an ipsilateral paralytic clubfoot. She was treated with single-stage excision of constriction band and Z-plasty. Spontaneous union of the femur was achieved at 3 months. Procurvatum deformity of the femur improved gradually over 3 years. This happens to be the first and only reported case of congenital pseudarthrosis of the femur with sciatic nerve palsy due to congenital constriction band.     

Pancreatic solid pseudopapillary neoplasm

Pancreatic solid pseudopapillary neoplasm (SPN) is a rare low grade malignant tumour. Distinguishing this entity from other pancreatic neoplasms is critical for therapeutic decision making and prognostication. It predominantly affects young female patients <40 years of age, with excellent clinical outcome following surgical removal. The gold standard diagnostic test is cytopathological or histopathological assessment of fine needle aspirate. There are two main difficulties with this. First, SPN can present with morphological and immunohistochemical appearances that can closely mimic other pancreatic tumours, in particular, pancreatic neuroendocrine tumour (NET). Second, the amount of diagnostic material from fine needle aspiration can be limited. Here, we present a cytopathological case with both challenges during the pre-operative investigation of SPN. The case exemplifies the importance of combining morphological features with a targeted panel of immunohistochemistry to arrive at the diagnosis.     

An update on atypical large glandular proliferations of the prostate

The differential diagnosis of prostatic atypical large gland proliferations includes several benign and malignant entities. This review focusses on issues relevant to the practising pathologist, particularly around areas of controversy such as high-grade prostatic intraepithelial neoplasia (HGPIN) and intraductal carcinoma of the prostate (IDCP). HGPIN is a putative precursor of prostate cancer, but its clinical relevance is as a surrogate marker of unsampled prostate cancer, thereby identifying patients who would benefit from a prompt repeat biopsy. The incidence of missed prostate cancer is much lower in contemporary practice due to pre-biopsy MRI and extended sampling protocols so HGPIN is currently less important. It is however important to distinguish HGPIN from PIN-like carcinoma and IDCP. PIN-like carcinoma is considered a histological subtype/variant of acinar prostate carcinoma and should be graded as Gleason pattern 3. A diagnosis of cribriform HGPIN should not be made in needle biopsies as such a proliferation may represent IDCP. This review discusses controversies related to the diagnosis, reporting and management of IDCP. A personalized approach to management of patients with isolated IDCP in needle biopsies that is based on the histological and radiological features of an individual case is outlined.     

Three rare cases of benign mixed tumour of the skin displaying mature bone and cartilage elements

Mixed tumour of the skin is a rare adnexal tumour of sweat gland origin, which can be benign or malignant. Histologically, it is defined by the presence of epithelial, myoepithelial and mesenchymal elements. The latter can take several forms, but both bone formation and cartilage formation are independently rare features. We present three rare cases of mixed tumour of the skin. Two of these displayed both mature bone and cartilaginous mesenchymal elements, while the third displayed mature bone formation without cartilage.