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Objective: Unusual clinical course Background: Hypersplenism, the rapid and premature destruction of blood cells, encompasses a triad of splenomegaly, cytopenias (anemia, leukopenia, or thrombocytopenia), and compensatory bone marrow proliferation. Secondary hypersplenism results from non-intrinsic splenic diseases, such as hemoglobinopathies. Sickle cell disease consists of a group of genotypes, where hemoglobin sickle C disease (HbSC) is the inheritance of hemoglobin S with hemoglobin C. Most homozygous genotypes undergo complete auto-splenectomy by age 6 years, whereas those with HbSC disease rarely do. We report a rare case of hypersplenism and massive splenomegaly in an adult with sickle cell disease, the HbSC genotype, requiring splenectomy. Case Report: A 41-year-old woman with known splenomegaly initially presented to the general surgery clinic for management of abdominal pain. She was found to have anemia, indicating cytopenia likely from hypersplenism. Consequently, she underwent splenic artery embolization, followed by an exploratory laparotomy and splenectomy, with an unremarkable postoperative course. Conclusions: Acute splenic sequestration crisis can result from hypersplenism, a potentially fatal complication of sickle hemoglobinemia. The continuous cycle of sickled cell entrapment and stasis causes numerous splenic infarctions, forming splenic parenchymal scar tissue which reduces the spleen’s size and functionality – the process of auto-splenectomy. Adults rarely experience these crises past adolescence, which are secondary to the scarring and atrophy from premature auto-splenectomy. Our patient’s spleen measured 21.1 cm, larger than the average adult’s spleen. In our case, adjunctive preoperative splenic artery embolization likely contributed to decreased intraoperative blood loss during splenectomy, mitigating the need for perioperative transfusions. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Amyloidosis results in fibrillar sheets of beta-pleated amorphous congophilic protein deposition in the extracellular space. Breast amyloidosis is a rare entity, with the first case reported in 1973 and only 2 major case series published since. These deposits can have local or systemic manifestations and typically present unilaterally, although bilateral involvement has been described. Some reported cases of amyloidosis have been linked to breast cancer. Case Report: The patient was a 60-year-old woman who presented to the breast surgery clinic for evaluation after imageguided biopsy of a right breast lesion. Core needle biopsy under stereotactic guidance demonstrated pathology consistent with nodular deposition of amyloid, associated with calcifications. Microscopic examination revealed extracellular deposition of acellular eosinophilic material in fat, stoma, and blood vessels. Congo red special stain was positive. Amyloid with Congo red special stain showed apple green birefringence under polarized light. Surgical excision under needle localization was performed, with the final surgical pathology report confirming amyloid deposits. Conclusions: Breast amyloidosis can have calcium affinity, create a foreign body-like reaction with giant cell infiltration, and distribute through periductal, perivascular, or intralobar patterns. Some factors that can contribute to an increased risk or are associated with breast amyloidosis are predisposing clinical conditions, including autoimmune disease, various breast cancers, and B-cell lymphomas. Amyloidosis of the breast should be treated either as primary or secondary, based on etiology. Further studies need to be conducted on whether there are specific genetic markers that predispose populations to the development of amyloidosis of the breast. [ABSTRACT FROM AUTHOR]

Urinary bladder with concurrent colonic melanoma is an exceptionally uncommon occurrence, posing a diagnostic challenge for clinicians. While rare, it warrants consideration as a potential differential diagnosis, particularly in patients without a history of melanoma who present with persistent hematuria due to its aggressive nature. We present a case of a 55-year-old female with malignant melanoma involving the colon and urinary bladder presenting with hematuria. Given the scarcity of cases and variability in clinical management approaches, there is a pressing need for research efforts to establish standardized protocols and conduct trials to guide clinical practice in this rare entity. [ABSTRACT FROM AUTHOR]

Introduction: Mesenteric fibromatosis (intra-abdominal desmoid tumor) is rare, with only a few cases reported in the literature. Clinical symptoms range from asymptomatic, nausea, early satiety, abdominal pain, and gastrointestinal bleeding. Although histologically benign, such a tumor may become locally invasive, and aggressive forms contribute to significant morbidity and mortality. Case Presentation: We report the case of a 52-year-old West African male with a 1-year history of intermittent hematochezia and intermittent bloating. Colonoscopy revealed a 4-mm rectal polyp and internal hemorrhoids. Esophagogastroduodenoscopy revealed a severe duodenal stricture 4–5 cm distal to the ampulla. Further work-up with contrast-enhanced computed tomography of the abdomen and pelvis revealed a 5.0 × 3.7 × 4.3-cm mass within the mesentery, encasing the distal portion of the duodenum. Exploratory laparotomy was performed, and the mass was excised from the jejunum. Histopathology findings and immunohistochemical analysis revealed the diagnosis to be mesenteric fibromatosis (desmoid tumor), positive for nuclear β-catenin and SMA, and negative expression of STAT6, desmin, caldesmon, pan-cytokeratin, or c-KIT. The Ki67 index is <1%. Conclusion: This case report highlights the diagnostic challenges of mesenteric fibromatosis due to its nonspecific clinical presentation. Recognizing uncommon presentations of mesenteric fibromatosis and risk factors aids in early diagnosis, management, and treatment. Importantly, this also aids in the prevention of complications such as intestinal obstruction, bowel ischemia, and fistula formation. [ABSTRACT FROM AUTHOR]

B-cell lymphoma is a lymphoproliferative non-Hodgkin lymphoma arising from B cells, a type of immune lymphocytes that produces antibodies in the follicles of lymph nodes. Primary cutaneous B-cell lymphoma (PCBCL), a subtype of B cell lymphoma, originates within cutaneous tissue without evidence of extracutaneous involvement. There are very few reports of PCBCLs originating in the scalp. The most common tumors of the scalp are usually benign with only 1%–2% being malignant, most being basal cell carcinoma, squamous cell carcinoma, or melanoma. Primary cutaneous follicular cell lymphoma (PCFCL) is regarded as the most common lymphoma of the skin with an indolent course and favorable prognosis due to the response rate to treatment methods such as surgical removal with local radiotherapy, topical drugs, and intralesional therapies. This report highlights a rare case of PCFCL originating in the scalp, to raise awareness of a topic that requires continued established management. [ABSTRACT FROM AUTHOR]

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. (Arch Pathol Lab Med. 2015; 139:819-822; doi: 10.5858/arpa.2013-0358-RS), Metaplastic carcinoma of the breast (MCB) was first described in 1973 by Huvos et al (1) and was defined as a mammary carcinoma with mixed epithelial and sarcomatoid components. The [...]

Abstract Background Intraductal 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 presentation Patient 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. Conclusion Adenocarcinoma of ducts of Luschka should be considered among differential diagnoses for the patients with typical clinical presentations of chronic cholecystitis and cholelithiasis.

Abstract Background Neuroendocrine 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 presentation We 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. Discussion There 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. Conclusion Wide 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.

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. (Arch Pathol Lab Med. 2013;137:1152-1154; doi: 10.5858/arpa.2012-0294-RS), Primary effusion lymphoma (PEL) was first recognized as a distinct entity in the World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues in 2001. (1) It [...]

Cytokeratin (CK) 7 is normally expressed in the vast majority of lung adenocarcinoma (ADC). However, on rare occasions, as reported in this paper, CK7 negativity can challenge the diagnosis of pulmonary ADC. Hence, the need to use a combination of 'immunomarkers' such as thyroid transcription factor 1, Napsin A, p40, p63 and CK20. [ABSTRACT FROM AUTHOR]

Glomus tumors are uncommon, benign lesions commonly located on the digits of the hands and are diagnostically challenging. This is because hemangiomas or ganglion cysts are more commonly identified in those locations. Our case report underlines the diagnostic challenge of a glomus tumor and the importance of immunohistochemical staining. [ABSTRACT FROM AUTHOR]

* 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., Rosen described syringomatous adenoma of the nipple (SAN) in 1983. SAN is morphologically similar to syringoma, a tumor of eccrine duct origin. Although "derivation from eccrine structures of the nipple" [...]