Colonic Goblet Cell Carcinoid: Rarity of a Rarity! A Case Report and Review of Literature.

Goblet cell carcinoids (GCC) are extremelyrare neuroendocrine tumours, and characterised by their unique combination of two types of cancer cells âÃÂ" neuroendocrine (carcinoid) and epithelial (adeno-carcinoma). In spite of the fact that GCC is regarded as Neuro-Endocrine Tumour (NET), it does not illicit carcinoid syndrome. GCC usually arises in the appendix and accounting for less than 14% of all appendiceal tumours.Primary extra-appendiceal GCC have been reported as stomach, duodenum, small intestine, colon and rectum. The paper presents a rare case of GCC of the ascending colon in a 57-year-old male.

Crusted scabies in a renal transplant recipient treated with daily ivermectin: A case report and literature review.

Crusted scabies is a rare disease variant associated with T-cell dysregulation. Transplant patients are at risk of developing crusted scabies as a consequence of their immunosuppressive regimens. We report a case of crusted scabies presenting with recurrent septicemia in a 65-year-old renal transplant recipient, treated with daily ivermectin for 7 days after initial failure of weekly ivermectin dosing. A literature review of crusted scabies in transplant recipients consisting of 19 cases reports was summarized. Pruritus was common, and initial misdiagnosis was frequent. Most were treated with topical therapy, with one-third receiving ivermectin. Three of seven cases presenting with a concomitant infection died. Crusted scabies is commonly misdiagnosed in transplant recipients owing to its rarity, varied appearance, and different skin distributions. It should be considered in the differential diagnosis of transplant recipients presenting with rash and pruritus, given its association with secondary infection and subsequent mortality.

Intra-articular solitary fibrous tumor of the knee.

A rare case of intra-articular solitary fibrous tumor of the knee in an 84-year-old man is presented. This case report illustrates that solitary fibrous tumor should be included in the extended differential diagnosis of an intra-articular soft tissue mass.

Primary cutaneous versus salivary gland origin mucoepidermoid carcinoma.

Mucoepidermoid carcinoma (MEC) is a malignant glandular epithelial neoplasm that most commonly arises in the major salivary glands. Primary cutaneous MEC is very rare. There is a particular diagnostic challenge in determining the primary site of MEC when it is found in skin overlying the parotid gland. Attention to a combination of morphologic findings may be helpful. However, differentiation of primary cutaneous MEC from secondary cutaneous involvement by a parotid MEC may be unnecessary once the parotid gland is infiltrated. We report the case of a 54-year-old male with a 2-cm asymptomatic mass overlying the right parotid gland, which was managed by excision of the affected skin, right total parotidectomy, and right neck dissection. Histopathologic and immunohistochemical findings were consistent with a cutaneous intermediate-grade MEC. Postoperative radiotherapy was deferred. The patient showed no evidence of recurrence or metastasis at 2 months before self-discontinuing follow-up. To our knowledge, this is only the second reported case of MEC involving the parotid gland, but of overlying primary cutaneous origin.

Signet-ring cell melanoma: a potential diagnostic pitfall.

Malignant melanoma is commonly known as the great mimicker and can present in various clinical manifestations and with diverse morphological variants. One of the rare histological variants is the signet-ring cell type. The signet-ring morphology has been reported in numerous other neoplasms including adenocarcinoma, lymphoma, liposarcoma, squamous cell carcinoma, and basal cell carcinoma. We report a rare case of primary signet-ring cell malignant melanoma in a 62-year-old man. He initially presented with an enlarging nevus on his right flank with surrounding erythema. A biopsy showed atypical epithelioid and signet cells with prominent nucleoli and occasional mitoses. Initial diagnosis favored metastatic signet-cell carcinoma of gastrointestinal origin. Review of the biopsy and immunohistochemical analysis revealed the malignant signet-ring cells stained with S100, vimentin, and melanoma cocktail, in keeping with melanoma. The signet-cell morphology can be found in a variety of other malignancies. To prevent potential misdiagnoses, thorough histological examination should be aided by an appropriate immunohistochemical panel to confirm melanoma and exclude erroneous differentials.

Unique Case of Parathyroid Adenoma With Arteriovenous Malformation.

Direct communication between dysmorphic arteries and veins without an interceding capillary segment is known as arteriovenous malformation (AVM). Its etiology is still unknown; however, it is commonly acknowledged that it could be related to trauma or is congenital in origin. Often, AVMs are found in the central nervous system or other sites such as under the skin or in the deep solid organs. They can be encountered as a solitary abnormality or associated with another pathology. If they are large enough, they can deprive the neighboring tissue of oxygen, eventually leading to tissue damage and compressing the surrounding organs, causing potentially more serious consequences. AVM in parathyroid adenoma is an unusual entity in the medical reports and known clinical practice. We herein report a unique case of a 49-year-old female patient who presented with a neck mass and associated symptoms of hyperparathyroidism (HPT) with no history of previous trauma or surgery. The imaging and laboratory tests were consistent with parathyroid neoplasm. Parathyroidectomy was performed and revealed parathyroid adenoma with AVM.

Gastric Schwannoma as an Important and Infrequent Differential Diagnosis of Gastric Mesenchymal Tumours: A Case Report and Review of Literature.

The spectrum for gastrointestinal tract mesenchymal tumours includes leiomyomas, leiomyosarcomas, gastrointestinal stromal tumours (GISTs) and schwannomas. Schwannomas (also known as neuroma, neurilemmomas or neurinomas of Verocay) are well-known slow-growing, benign neoplasms that originate from nerve plexuses within a Schwann cell sheath. They can arise anywhere along the course of the peripheral nerve and are frequently reported around the head and neck, brachial plexus and along the gastrointestinal tract. Usually, these tumours are detected as solitary; however, they can occur at multiple sites around the body. Schwannomatosis (multiple schwannomas) is usually associated with neurofibromatosis type 2; the pathogenesis is triggered by mutations of the neurofibromatosis 2 tumour suppressor gene resulting in a loss of its function. Solitary gastric schwannomas are rare lesions that arise from the nerve plexus of the gastric wall. Frequently they are detected incidentally or may present with nonspecific abdominal pain or bleeding. This paper reports the case of a 79-year-old patient diagnosed with gastric schwannoma after presenting with abdominal pain. Gastric schwannomas should be taken into consideration while making a differential diagnosis of lesions that are gastric mesenchymal tumours, which span a broad spectrum. Gastric schwannomas are typically benign, considerably less common than gastric GISTs, and have an excellent prognosis following excision.

Signet ring squamous cell carcinoma--the forgotten variant: case report and review of the literature.

Squamous cell carcinoma is one of the commonest skin cancers. In most instances, histological diagnosis is straightforward and does not pose any diagnostic challenge to the handling pathologist. We report a rare case of signet ring squamous cell carcinoma (SRSCC) occurring in an 84-year-old woman who presented with a sore on the upper lip. Excision of the lesion showed signet ring carcinoma within a scar and surface ulceration. Deeper sections showed focal area of squamous differentiation. Although rare, consideration of squamous cell carcinoma as a diagnosis of primary skin tumors with signet ring morphology, especially in the absence of history of adenocarcinoma, may prevent missing rare cases of SRSCCs.

Intra-articular nodular fasciitis of the shoulder: a case report and review of the literature.

Nodular fasciitis is a benign proliferation of myofibroblasts usually arising adjacent to the fascia. In this report, we describe a rare case in which nodular fasciitis occurred in an intra-articular location in the shoulder of a 26-year-old man. The mass developed in the subscapularis recess of the shoulder and histological evaluation showed a myofibroblastic proliferation. MRI findings of intra-articular nodular fasciitis are discussed along with a review of previous reports.

A brief report of a rare case of giant apocrine hidrocystoma presenting as a scalp hematoma.

Apocrine hidrocystoma is a benign cystic tumour. The head and neck region is the site of predilection; occurrence in the scalp is extremely rare. We report a rare case of giant apocrine hidrocystoma with an unusual presentation as scalp haematoma.

Russell Body Typhlitis: A Case Report and Literature Review.

Russell bodies are accumulation of immunoglobulin in plasma cells forming intracytoplasmic inclusions. Russell body colitis is rare with only 3 cases described in the English literature up to date. We report a 78-year-old male with cirrhosis showing prominent cecal infiltration of Russell body containing plasma cells. Plasma cells showed no nuclear atypia or mitoses, and no evidence of light chain restriction. In this article, we report a fourth case of Russell body colitis, that is unique in being localized to the cecum in contrast to the other 3, 1 of which was in an inflammatory polyp in the sigmoid colon, 1 in a rectal tubulovillous adenoma and 1 as part of diffuse gastrointestinal disease. This is therefore the first report of localized Russell body typhlitis, occurring in a cirrhotic patient in whom an adjacent erosion was likely nonsteroidal anti-inflammatory drug-associated, a combination that may have facilitated the formation of Russell bodies.

Malignant giant cell tumor of the vulva.

Giant cell malignant fibrous histiocytoma or giant cell tumor of the soft parts (GCTSP) is a rare soft tissue tumor. GCTSP has an unpredictable behavior; the majority of the reported cases showed benign histology and those that showed malignant morphologic features were extremely rare. To the best of our knowledge, GCTSP has never been reported to involve the vulva in the English literature. We report the first case of malignant GCTSP of the vulva. Histologic features and the immunoprofile of the tumor and differential diagnosis are discussed in detail. Although extremely rare, consideration of the potential occurrence of GCTSP in the vulva is recommended to avoid a potential diagnostic pitfall.

WITHDRAWN: Syringoma: the clear cell variant made clearer.

This article has been withdrawn at the request of the author(s) and/or editor. The Publisher apologizes for any inconvenience this may cause. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.

Cellular variant of extraskeletal myxoid chondrosarcoma with Ewing's sarcoma-like areas: a diagnostic pitfall in core needle biopsy.

Extraskeletal myxoid chondrosarcoma (EMC) is a rare mesenchymal soft tissue tumour that poses diagnostic difficulty as it lacks a characteristic immunophenotype, in addition to its wide morphological spectrum. Microscopically, EMC shows strands and cords of relatively small cells with acidophilic cytoplasm that are occasionally vacuolated. Small cells with scant cytoplasm may comprise some EMC. We describe a rare and challenging case of EMC, which shows an unusual morphology with small blue cells, raising the possibility of PNET/Ewing's sarcoma. The small cellular variant of EMC usually poses diagnostic difficulty, particularly during its evaluation in a core needle biopsy. Consideration of EMC, small cell variant, in evaluation of a blue cell tumour, may avoid a potential diagnostic pitfall. Proper diagnosis of EMC and its differentiation from PNET/Ewing's sarcoma is crucial due to a difference in management protocols and prognostic outcome.

Enteric type urachal adenocarcinoma: a case report.

Urachal pathology is rare. The most frequently reported lesion is urachal adenocarcinoma. The pathogenesis of urachal adenocarcinoma is unknown. We report a case of a 55-year-old man who presented with microscopic hematuria. Clinical work up showed a tumor involving the urinary bladder with extravesical extension. Masses or tumors involving other organs were excluded. Partial cystectomy revealed a distended bladder wall with the formation of a cystically dilated mass filled with mucoid material. Microscopic examination showed enteric type adenocarcinoma with abundant mucin formation. The neoplastic urachal epithelium showed features of colonic differentiation as evidenced by the presence of goblet cells and positive staining for acid mucin and cytokeratins 20 (CK 20). Such features are absent in non-neoplastic urachal epithelium. This was a rare case of urachal adenocarcinoma, enteric type, with abundant mucin formation. The urachal adenocarcinoma had morphological features and an immunohistochemical profile that were similar to that of adenocarcinoma of the colon.

An updated review on the clinicopathologic aspects of arrhythmogenic right ventricular cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) is a disease that affects young adults and perhaps is the most common cause of sudden death in this age group. Many of these cases are faced by forensic pathologists, without a prior history, and unless suspected by the pathologist at the time of autopsy, the diagnosis is likely to be missed. Since some of these cases are hereditary, consequences of missing the diagnosis have a medicolegal implication that extends to involve other family members of the deceased. ARVC raises many controversial issues with regard to its existence, defining and diagnostic criteria, pathologic spectrum of changes, and pathogenesis. We reviewed the recent literature and included our experience to dictate a novel approach to the inherent problems faced by pathologists. In this review, we identified the most characteristic and distinct histopathologic features that are diagnostic or highly suggestive of ARVC, even in the absence of clinical history. We also highlighted the new insights on the disease pathogenesis. Hence, this review provides a better understanding of the disease and sheds light on many controversial issues regarding ARVC.

Clear cell hidradenoma: an unusual vulvar tumor.

Clear cell hidradenoma (CCH) is a benign skin appendageal tumor. Most cases occur in the skin of the head and face. The tumor usually presents as a solitary firm dermal nodule. Histologically, it is a well-circumscribed, nonencapsulated tumor formed of 2 cell types, one with clear cytoplasm and the other with dark eosinophilic cytoplasm. Although most cases are benign, occasional cases with malignant transformation have been reported. We report a case of CCH in a very unusual location. To the best of our knowledge, CCH has not been previously described in this site, in the English literature. Although rare, inclusion of CCH should be included in the differential diagnosis of benign solid and clear cell tumors of the vulvar skin.

Sebaceoma of the external ear canal: an unusual location. Case report and review of the literature.

Sebaceous neoplasms of the external ear canal are extremely rare. Only two cases of sebaceous neoplasms have been reported in the English literature, a sebaceous carcinoma and a sebaceous adenoma. We report a case of sebaceoma of the external ear canal. To the best of our knowledge, sebaceoma of the auditory canal has not been reported previously. We highlight the differential diagnosis, particularly sebaceous carcinoma and basal cell carcinoma with sebaceous differentiation. Awareness of the possible occurrence of sebaceoma in the auditory canal may prevent the diagnostic pitfall of misidentifying this tumor.