Cutaneous Angiomyolipoma of the Ear: A Case Report and Literature Review.

Cutaneous angiomyolipoma is a rare mesenchymal tumor, distinct from its renal counterpart. Only few cases have been reported in English language literature to date. Here, we report a case in a 36-year-old man who presented with a painless swelling on his right ear. Microscopy revealed a well-circumscribed tumor, composed of an admixture of mature adipocytes, blood vessels, and bundles of smooth muscles. The tumor cells were positive for desmin and calponin but negative for HMB45, melan-A, SOX10, tyrosinase, and CD10. We present the case to emphasize the uniqueness of cutaneous angiomyolipoma from its renal counterpart. We also review the relevant literature on this topic.

Spectrum of findings in orchiectomy specimens of persons undergoing gender confirmation surgery.

Gender confirmation surgery is increasingly common in persons with gender dysphoria. We describe changes seen in gonads from individuals seeking male-to-female physical adaptation. We studied 99 orchiectomies from 50 persons. The average age was 33 years (range, 21-63 years). Eighty-six (86.8%) of 99 testes were normal in size with an average size of 3.87 cm (range, 3.0-5.5 cm). Thirteen (13.1%) of 99 testes were hypotrophic and measured up to 2.5 cm. Seminiferous tubules were reduced in diameter compared with controls (0.137 mm versus 0.237 mm; P < .001) and showed peritubular fibrosis in 41 (82%) of 50 persons. In 40 (80%) of 50 persons, there was maturation arrest at the spermatogonia level. In 10 (20%) of 50 persons, the seminiferous tubules showed focal spermatids/spermatozoa up to 7 per 10 tubules mixed with partial maturation arrest at primary spermatocytes. Twenty-six (26%) of 99 testes showed seminiferous tubules with rare cells with large nuclei (3× size of Sertoli cells nuclei) and degenerative chromatin (cytomegaly). Leydig cells were absent in 50 (50%), markedly reduced in 30 (30%), and similar to controls (mean, 33/high-power field) in 20 (20%). A subset (20/99; 20%) of testes had epithelial hyperplasia of the proximal epididymis with stratification and micropapillae. There was no germ cell tumor, sex cord stromal tumors, or germ cell neoplasia in situ. In summary, the histologic changes include (1) decreased diameter of seminiferous tubules and expansion of the interstitium, (2) marked hypoplasia of germ cells, (3) rare cytomegaly, (4) hypoplasia or absence of Leydig cells, and (5) epididymal hyperplasia.

Effective use of Twitter and Facebook in pathology practice.

Social networking has become a part of daily life for most people. In the era of globalization, it has become indispensable for effective communication between friends and peers across the world. Today, social media use is not restricted only to our personal lives; it is becoming increasingly incorporated into our professional lives, as well. Social media platforms are rapidly becoming a necessary tool for medical professionals. Apt use of social media platforms can revolutionize scientific communication and collaboration. Pathologists are increasingly counted among Facebook's 2 billion users and Twitter's 328 million members. Social media can be a powerful tool not only for individual career progression but also for raising the public profile of the specialty of pathology. It is now possible for us to reach out to millions of people, simultaneously and instantaneously, just by touching a screen. Real-time interactions between physicians, across geographic borders and subspecialty borders, will undeniably open unexplored avenues for effective communication, consultation, research collaboration, and education. Knowledge sharing through social media will immensely enrich and grow our careers as pathologists; it will also enlighten the public and physicians of other specialties as to the crucial role of pathologists in patient care. We discuss the uses of Facebook and Twitter in modern day pathology, its implication in routine daily practice, pros and cons, and tips for effective social media use by pathologists.

Case Study: Rare Case of Mantle Cell Lymphoma With Extranodal Involvement in the Foot.

Mantle cell lymphoma (MCL) is a rare type of non-Hodgkin lymphoma that commonly affects extranodal sites. The most commonly affected sites are the bone marrow, gastrointestinal tract, Waldeyer's ring, lung, and pleura. We report the case of an 80-year-old diabetic male, in MCL remission, who presented with a small dome-shaped nodule on his calf and an ipsilateral second digit non-healing ulceration after a traumatic fall. Despite surgical and conservative treatment, the wound worsened, resulting in histopathologic examination, which confirmed the presence of lymphocytes, indicating MCL relapse. This case was followed up for approximately 3 months until the patient died. Our case is an example of pedal manifestations of skin involvement of MCL, which, on consideration of the clinical manifestations also, can be confused with a nonhealing diabetic wound. The clinical significance of our case study is to assist in the diagnosis, management, and prognosis of a patient with MCL.

Ileal Pouch Biopsy Triggers Investigation and Diagnosis of Systemic Mastocytosis.

We report a unique case of systemic mastocytosis (SM) diagnosed in an ileal pouch biopsy obtained from a 44-year-old woman with ulcerative colitis. She presented with intermittent abdominal pain and watery diarrhea that did not respond to antibiotic therapy. The pouch biopsy showed expansion of the lamina propria by aggregates of CD117 and CD25-positive abnormal mast cells. A subsequent bone marrow analysis showed an increase in abnormal mast cells. Based on World Health Organization criteria, she was diagnosed with SM and responded to cromolyn sodium therapy. Systemic mastocytosis can mimic pouchitis, and thus recognition of this condition is important for appropriate clinical management.

Very delayed cervical lymph node metastases from hyalinizing clear cell carcinoma: report of 2 cases.

BACKGROUND: Hyalinizing clear cell carcinoma (HCCC) is a rare salivary gland neoplasm most often found in the oral cavity. Although it is generally a low-grade malignancy that is treated with wide local excision, there is a growing body of evidence pointing toward the potential for more aggressive behavior. METHODS: We reviewed available records of patients with delayed cervical lymph node metastases from HCCC. RESULTS: Two patients who were treated with wide local resection for HCCC and remained disease-free at the primary site were diagnosed with cervical lymph node metastases 10 and 14 years later. We treated both with neck dissection, and 1 patient received adjuvant radiation therapy. CONCLUSION: These cases illustrate the risk for occult nodal metastases from HCCC with delayed presentation. Clinician awareness of the presence of subclinical metastases in the neck requires thorough long-term surveillance and potential intervention should nodal disease become manifest.

Mixed adenocarcinoma-carcinoid (collision tumor) of the appendix.

OBJECTIVE: To report a case of mixed adenocarcinoma and carcinoid in the gastrointestinal tract. CLINICAL PRESENTATION AND INTERVENTION: A 52-year-old man presented with a mass at the base of the appendix. On microscopic examination of the tumor, mixed adenocarcinoma and carcinoid was identified. The carcinoid component was insular/trabecular in morphology, and the epithelial component revealed mucinous adenocarcinoma. Morphologically and immunohistochemically the 2 components showed a clear-cut distinction, without any intervening intermediate cell population. The adenocarcinomatous component displayed omental deposit and metastasis to the regional lymph nodes. One year later, the patient developed multiple metastatic deposits of adenocarcinoma in the liver, rectus muscle, ileum and sigmoid colon. CONCLUSION: This tumor had an aggressive clinical course and behaved more like adenocarcinoma than carcinoid.

Jejunal perforation caused by schistosomiasis.

We report an extremely unusual case of jejunal perforation caused by adult schistosoma worm in a 49-year-old South Korean man who presented with acute abdomen. To our knowledge, this is the first case of jejunal perforation associated with adult worm of schistosoma.

Phyllodes tumor of the vulva: Report of a rare case and review of the literature.

Phyllodes tumor occurring in the vulva is extremely rare; only 6 cases have been previously reported in the literature. The histogenetic origin of this tumor is controversial as it is being debated whether such lesions evolve from ectopic breast tissue, cutaneous apocrine glands, and most recently, anogenital mammary-like gland. We report an 18-year-old Kuwaiti girl who presented with a painless mass in the left labium majus, which was subsequently excised. Microscopic examination revealed morphologic pattern characteristic of benign phyllodes tumor. Immunostaining demonstrated the presence of estrogen receptors and progesterone receptors in the epithelial component. We present this case to emphasize the importance of recognizing this uncommon lesion occurring at an extremely unusual site. We also discuss the histogenesis of phyllodes tumor and related lesions occurring in the anogenital region in light of the current literature along with a brief review of the previously reported cases of vulvar phyllodes tumor.

Proximal-type epithelioid sarcoma in the groin presenting as a diagnostic dilemma.

Epithelioid sarcoma is an uncommon soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in the distal extremities of young adults. Lately, a 'proximal' subtype has been described, which occurs in the pelvic and genital areas of somewhat older individuals and tends to behave more aggressively than the conventional subtype. The correct diagnosis of this subtype is essential, since this tumor can be easily mistaken for other malignant tumors that exhibit epithelioid morphology. We report a case of proximal-type epithelioid sarcoma that presented as an inguinal mass in a 47-year-old man. Histologically, the tumor consisted of diffuse sheets of epithelioid cells with scattered rhabdoid morphology. By immunohistochemistry, the neoplastic cells expressed cytokeratin, epithelial membrane antigen, vimentin, CD34, CD99 and showed complete loss of nuclear INI1 protein expression. Fluorescence in situ hybridization was considered borderline for 22q deletion. We present this case to emphasize the importance of diagnosing this uncommon tumor and the role of INI1 immunohistochemistry in establishing the diagnosis.

An unusual case of extensive epididymotesticular malakoplakia in a diabetic patient.

A 67-year-old Saudi man with poorly controlled diabetes-mellitus underwent orchiectomy for a painful left testicular swelling that did not respond to antibiotics. Clinical diagnosis was testicular malignancy. Histologic workup demonstrated extensive involvement of the testis and epididymis by diffuse infiltrate of large histiocytes with eosinophilic granular cytoplasm and numerous Michaelis-Gutmann bodies, which were diagnostic of malakoplakia. Very rarely, malakoplakia has been described in association with diabetes mellitus. Such an extensive malakoplakia occurring at an unusual site in association with systemic illness lends support to the view that a compromised immune status could be etiologically important in malakoplakia. We present this case to highlight the importance of diagnosing malakoplakia when it occurs at unusual locations. We also explore the role of diabetes mellitus in the pathogenesis of malakoplakia and compare the relationship between malakoplakia and xanthogranulomatous inflammation, which are closely related to each-other on both clinical and morphologic grounds.

Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease): evidence for a dendritic cell derivation?

Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease is a rare histiocytic disorder of unknown origin. Both dendritic cell and monocyte-macrophage lineage have been considered as the origin of the histiocytes that are classical of the disorder. We analyzed seven cases of Rosai-Dorfman disease to determine the immunophenotypic profile of these cells. Immunohistochemical analysis was undertaken by using: (a) fascin as a novel mature dendritic cell marker; (b) S-100 protein; (c) CD68 (both KP1 and PGM1) as macrophage related markers; (d) CD79a and (e) CD3. The Rosai-Dorfman histiocytes exhibited strong cytoplasmic staining for fascin in all the cases and demonstratedformation of a meshwork of fine dendritic processes emanating from the cell cytoplasm. S-100 protein was uniformly expressed in all the cases. Expression of both KP1 and PGM1 epitopes of CD68 was found to be weak and variable. The phagocytosed lymphocytes were composed of an admixture of both B and T-lymphocytes. The characteristic expression pattern of the histiocytes for fascin along with co-expression of S-100 protein and a consistently weaker and variable expression of macrophage-lineage markers point more towards a dendritic cell ontogeny of these cells. Further, abundant fascin expression and presence of dendritic processes indicate a differentiated or mature dendritic cell phenotype for these cells.

Infantile fibromatosis of the nose and paranasal sinuses: report of a rare case and brief review of the literature.

Infantile fibromatosis is an uncommon, usually cutaneous, nonmetastasizing but locally aggressive fibroblastic proliferation. Involvement of the sinonasal area is extremely uncommon, and only 5 cases have been previously reported. We report a new case, which occurred in a 2-year-old boy. The mass involved the nasal cavity and the paranasal sinuses and extended into the cranial cavity. We also discuss the clinicopathologic features of this lesion, including its ultrastructural characteristics, and we review the literature.