Philip Xiao

Infiltrating Syringomatous Adenoma of the Nipple: Clinical Presentation and Literature Review

Infiltrating syringomatous adenoma of the nipple is a rare neoplasm of the breast. Syringomatous adenoma of the nipple is often misdiagnosed because clinical examination and mammographic findings of syringomatous adenoma of the nipple mimic carcinoma. Despite its benign behavior, syringomatous adenoma of the nipple usually shows infiltrative expansile proliferation into adjacent tissue and underlying breast tissue. Up until now, to our knowledge, there has been no reported case of regional or distant metastasis. Histologically and clinically, syringomatous adenoma of the nipple is often confused with tubular carcinoma as well as low-grade adenosquamous carcinoma of the breast. Special attention given to this tumor by pathologists and clinicians can avoid misdiagnosis and unnecessary treatment.

Primary Effusion Lymphoma

Primary effusion lymphoma is a large cell non-Hodgkin lymphoma localized predominantly in body cavities and occasionally in extracavitary regions. It presents with characteristic lymphomatous effusions in the absence of solid tumor masses, and pleural, peritoneal, and pericardial spaces are most often involved. It is typically associated with human herpesvirus 8 infection in immunocompromised individuals, in the setting of human immunodeficiency virus infection, organ transplantation, or in rare cases advanced age. Histologically, primary effusion lymphoma is characterized by atypical lymphoid cells of B-cell lineage with large nuclei and prominent nucleoli. Demonstration of human herpesvirus 8 latent antigens is required for diagnosis, and treatment modalities are limited at this time. In this review, we aim to summarize clinicopathologic features of this rare and unique entity.

Neuroendocrine (Merkel cell) carcinoma of the retroperitoneum with no identifiable primary site

BackgroundNeuroendocrine carcinoma is an aggressive neoplasm that mainly affects elderly Caucasians and typically arises in sun-exposed areas of the skin. The disease is rather rare and only a relatively few cases present with no apparent primary lesion.Case presentationWe report a case of an 81-year-old Caucasian male with neuroendocrine carcinoma, which initially presented as a large retroperitoneal mass. Pathological and immunohistochemical analysis of the transabdominal CT-guided biopsy specimen revealed tissue consistent with neuroendocrine carcinoma. The patient underwent exploratory laparotomy and the mass was successfully excised along with an associated mesenteric lymph node.DiscussionThere are currently two possible explanations for what occurred in our patient. First, the retroperitoneal mass could be a massively enlarged lymph node where precursor cells became neoplastic. This would be consistent with a presumptive diagnosis of primary nodal disease. Alternatively, an initial skin lesion could have spontaneously regressed and the retroperitoneal mass represents a single site of metastasis. Since Merkel cell precursors have never been identified within lymph nodes, the latter theory seems more befitting. Moreover, metastasis to the retroperitoneal lymph nodes has been reported as relatively common when compared to other sites such as liver, bone, brain and skin.ConclusionWide local excision of the primary tumor is the surgical treatment of choice for localized disease. We propose that further studies are needed to elucidate the true efficacy of chemotherapy in conventional as well as unconventional patients with neuroendocrine carcinoma.

Metaplastic Carcinoma of the Breast

Metaplastic carcinoma of the breast is a rare but aggressive type of breast cancer that has been recognized as a unique pathologic entity by the World Health Organization. Morphologically, it is characterized by the differentiation of neoplastic epithelium into squamous cells and/or mesenchymal-looking elements (squamous cells, spindle cells, cartilage or bone, etc). It shares many similarities with invasive ductal carcinoma and benign lesions on mammography, which further complicates the diagnosis. Treatment for metaplastic breast carcinoma is relatively unknown because of the rarity of the disease, but studies suggest that removal of the tumor and adjuvant radiation therapy has the greatest benefit.

Isolated intracranial Rosai-Dorfman disease with orbital extension.

Dear Professor Kaye, Sinus histocytosis with massive lymphadenopathy, or RosaiDorfman disease (RDD) is a rare non-neoplastic histiocytic disorder that was first described in 1969 by Rosai and Dorfman. This lymphoproliferative disorder of uncertain etiology that affects mainly children and young adults is characterized clinically by massive, painless cervical lymphadenopathy, fever, leukocytosis, elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia. Histopathologically, the disease is characterized by lymph node sinus dilation with emperipolesis, a distinctive feature whereby histiocytes phagocytize intact lymphocytes. Most patients with RDD present with nodal involvement, while a minority of patients (40%) have extranodal involvement. Interestingly, the disease can affect any system with a propensity for tissues of the head and neck, with nasal and oral cavities and paranasal cavities being affected in 10% to 20% of cases. However, isolated central nervous system (CNS) involvement of RDD is extremely rare (5–10%), with fewer than 100 reported cases to the best of our knowledge. CNS lesions have commonly involved the skull base with a leptomeningeal component. Neurologically, patients frequently present with headaches, seizures, numbness, paraplegia and visual disturbances. Radiographically, the lesions appear to be dural-based, extra-axial and enhancing with surrounding vasogenic edema. Therefore, intracranial RDD can closely mimic meningiomas, both neurologically and especially radiographically. We present a rare case of extranodal, intracranial RDD with orbital extension that was originally diagnosed preoperatively as meningioma.

Duodenal web associated with malrotation and review of literature

Intestinal obstruction due to midgut malrotation in neonates is well known. The incidence of malrotation in newborns is around 1:500 and the symptomatic incidence is 1:6000 births. Duodenal web as a cause of intestinal obstruction is less common and is reported to be 1:10 000–1:40 000. Malrotation is known to be associated with other congenital obstructive anomalies including duodenal atresia, stenosis and duodenal web. But, intestinal obstruction due to malrotation associated with duodenal web has been reported only rarely with a few published cases in our literature review. We present a case of intestinal obstruction diagnosed in the prenatal period via sonogram. A plain X-ray of the abdomen after birth showed a distended duodenum with paucity of air distally suggesting duodenal obstruction. An exploratory laparotomy showed a duodenal web proximal to the sphincter of oddi. The patient also had an associated malrotation and underwent Ladds procedure and appendectomy. The post-operative period was uneventful.

Ectopic Hepatic Tissue Presented as a Posterior Mediastinal Mass

We present a young patient with respiratory complaints that was found to have a mass in her right posterior mediastinum. The mass was diagnosed to be ectopic histologically unremarkable hepatic tissue. We have also reviewed several of the few intrathoracic ectopic liver cases in the literature, along with a brief discussion of the significance of such a finding.

Invasive ductal carcinoma of the breast with clear cell and pseudo-lactating changes

Invasive ductal carcinoma of the breast with clear cell changes is a very rare pathological entity. There are <150 cases of this breast cancer subtype reported in the literature. Clear cell carcinoma could be easily missed or under-diagnosed in a breast core biopsy specimen due to the fact that this subtype tends to show a papillary pattern with clear cell and pseudo-lactating changes, especially in young female patients. Normal breast tissue may possess clear cells as a consequence of the physiological changes of pregnancy, the clearing of the cytoplasm within myoepithelial cells, or the clearing of the cytoplasm in apocrine metaplasia. Owing to its aggressive clinical course, clear cell carcinoma must be differentiated from other subtypes of breast carcinoma. Special attention given to this rare subtype by pathologists and clinicians could avoid misdiagnosis and delay of treatment.

A Rare Presentation of Metastasis of Prostate Adenocarcinoma to the Stomach and Rectum

Prostate cancer is the second most common cause of cancer death in men in the United States. The most common sites of metastasis include the bone, lymph nodes, lung, liver, pleura, and adrenal glands, whereas metastatic prostate cancer involving the gastrointestinal tract has been rarely reported. A 64-year-old African-American man with a history of prostate cancer presented with anemia. He reported the passing of dark colored stools but denied hematemesis or hematochezia. Colonoscopy revealed circumferential nodularity, and histology demonstrated metastatic carcinoma of the prostate. Esophagogastroduodenoscopy showed hypertrophic folds in the gastric fundus, and microscopic examination revealed tumor cells positive for prostate-specific antigen. Bone scanning and computed tomography of the abdomen and pelvis did not show metastasis. It is crucial to distinguish primary gastrointestinal cancer from metastatic lesions, especially in patients with a history of cancer at another site, for appropriate management.

Intraductal and invasive adenocarcinoma of duct of Luschka, mimicking chronic cholecystitis and cholelithiasis

BackgroundIntraductal and invasive adenocarcinoma of duct of Luschka is rare. To the best of our knowledge, this is the second case report of intraductal and invasive carcinoma arising from ducts of Luschka.Case presentationPatient presented to hospital with signs and symptoms of chronic cholecystitis and cholelithiasis. Ultrasound examination revealed thickening of gallbladder wall with abnormal septation around liver bed. Patient underwent laparoscopic cholecystectomy and resection of the adjacent liver bed. Histologic examination confirmed an intraductal and invasive adenocarcinoma arising from Luschka ducts.ConclusionAdenocarcinoma of ducts of Luschka should be considered among differential diagnoses for the patients with typical clinical presentations of chronic cholecystitis and cholelithiasis.