Ocular cicatricial pemphigoid (OCP) is a chronic bilateral, blinding, cicatrizing form of conjunctivitis with relapsing and remitting periods. It has strong evidence for an immune type II hypersensitivity that leads to subconjunctival fibrosis and extensive systemic bullae formation. To the best knowledge of the authors, this is the first reported case of direct immunofluorescence (DIF) assay-proven OCP in an elderly Filipino man.

A 68-year-old male presented with bilateral corneal conjunctivalization, symblepharon, ectropion, conjunctival hyperemia testing positive with conjunctival biopsy for basement membrane antibodies with DIF for the left eye, while turning out negative for the right eye. He was managed as a case of OCP, both eyes, and was given topical steroids and antibiotics. Oral Dapsone was started by Dermatology and Rheumatology Services.

OCP is a rare autoimmune and blinding disease. Early diagnosis and prompt treatment are vital as ocular complications permanently affect the quality of life of patients as seen in our patient. DIF assay remains the gold-standard for diagnosis. Systemic immunosuppression is the mainstay of treatment. Adjunctive supportive topical medication may be given to alleviate ocular discomfort. A multidisciplinary approach is essential to provide holistic care to each patient.

Hyalinizing clear cell carcinoma of the salivary gland is a rare neoplasm, accounting for only less than 1% of malignancies arising from the salivary gland. It is molecularly defined by the expression of the EWSR-ATF1 fusion oncogene. To date, there has been no previous studies published yet in the Philippines regarding the existence of this tumor. In this paper, we present a case of a 70-year-old elderly female who had a 10-year history of a gradually enlarging left lateral neck mass. Histopathologic examination showed a tumor arranged of cords, nests, and trabeculae of monomorphic round cells with abundant clear to lightly eosinophilic cytoplasm surrounded by thick hyalinized collagen bundles. Immunohistochemistry and molecular studies were done which revealed a positive p63 staining, negative SMA and S100, and an EWSR1 rearrangement in Fluorescence in situ hybridization (FISH), thus, confirming the diagnosis.

BACKGROUND

Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL). Classification of DLBCL is often based on the cell of origin (COO), distinguishing between germinal center B-cell (GCB) and non-GCB subtypes. Although not yet recognized as a distinct entity by the World Health Organization (WHO), double expressor lymphoma (DEL), characterized by the co-expression of c-MYC and BCL2, carries an unfavorable prognosis for a subgroup of DLBCL patients. Another entity is the so-called high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements (double-hit/triple-hit lymphomas) diagnosed through fluorescent in-situ hybridization (FISH) analysis.

This study aimed to determine the clinicopathologic profile and survival outcomes of Filipino DLBCL patients at the Philippine General Hospital (2016-2021), comparing double-hit versus non-double-hit and doubleexpressor versus non-double-expressor lymphomas, and assessing concordance between FISH-measured double-hit and IHC-measured double-expressor statuses.

This is a single-arm, retrospective cohort study involving all surgical pathology cases signed out, with the aid of immunohistochemistry (IHC) studies, as NHL DLBCL, GCB, or non-GCB subtype, from 2016 to 2021. A second panel of IHC studies and FISH analysis using tissue microarray was subsequently done. Most cases exhibited a nonGCB subtype and were classified as DEL on second IHC panel. Five out of eleven DEL cases were reclassified as double hit lymphoma (DHL).

Clinically, most patients with these lymphomas present at age 60 years and below, exhibit B symptoms, with elevated serum lactate dehydrogenase (LDH) levels, at least stage III-IV disease at diagnosis, and possess a high International Prognostic Index (IPI) score, collectively indicating a poor prognosis.

Survival outcomes for patients with DLBCL ranges from three to 37 months. All cases of mortality were associated with DEL, contrasting with DHL cases which had variable outcomes. Due to limited sampling, statistical significance of the results cannot be determined. A comprehensive evaluation is essential to the diagnosis of DLBCL and DHL to include a complete immunohistochemistry panel and molecular testing, notably with FISH studies.

Gliomatosis peritonei (GP) is a condition characterized by the dissemination of mature glial tissues throughout the peritoneal cavity. It is usually associated with immature ovarian teratoma but presents with mature cystic teratoma (MCT) in 1% of cases. GP, associated with MCT, is a benign disorder. The majority of cases remain asymptomatic and rarely recur. Here, we present a case of a 22-year-old woman with a history of abdominal enlargement and severe abdominal pain who underwent exploratory laparotomy, peritoneal fluid cytology, bilateral salpingo-oophorectomy, appendectomy, omental biopsy, and Jackson-Pratt drain insertion with histopathologic result of GP with MCT. A month later, the patient had a recurrence of abdominal enlargement, necessitating a second surgery. Immunohistochemistry for histopathologic evaluation and diagnostic imaging are crucial in confirming the diagnosis and guiding the treatment strategy. A multidisciplinary team approach in monitoring and comprehensive support is significant in optimizing outcomes for patients with aggressive GPs associated with MCT. Further research and clinical experience are essential to establish a standardized guideline to improve the management and clinical outcome of this condition.

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare neoplasm of the kidney. Recognition of this rare entity is important with regards to a patient’s prognosis and therapeutic management.
Kidney Neoplasms ; Immunohistochemistry ; Pathology, Surgical

Humans ; Female ; Receptors, Estrogen/metabolism* ; Breast Neoplasms/metabolism* ; Receptors, Progesterone/metabolism* ; Immunohistochemistry ; Estrogen Receptor alpha

Background: Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL). Classification of DLBCL is often based on the cell of origin (COO), distinguishing between germinal center B-cell (GCB) and non-GCB subtypes. Although not yet recognized as a distinct entity by the World Health Organization (WHO), double expressor lymphoma (DEL), characterized by the co-expression of c-MYC and BCL2, carries an unfavorable prognosis for a subgroup of DLBCL patients. Another entity is the so-called high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements (double-hit/triple-hit lymphomas) diagnosed through fluorescent in-situ hybridization (FISH) analysis. Objective: This study aimed to determine the clinicopathologic profile and survival outcomes of Filipino DLBCL patients at the Philippine General Hospital (2016-2021), comparing double-hit versus non-double-hit and doubleexpressor versus non-double-expressor lymphomas, and assessing concordance between FISH-measured double-hit and IHC-measured double-expressor statuses. Methods: This is a single-arm, retrospective cohort study involving all surgical pathology cases signed out, with the aid of immunohistochemistry (IHC) studies, as NHL DLBCL, GCB, or non-GCB subtype, from 2016 to 2021. A second panel of IHC studies and FISH analysis using tissue microarray was subsequently done. Most cases exhibited a nonGCB subtype and were classified as DEL on second IHC panel. Five out of eleven DEL cases were reclassified as double hit lymphoma (DHL). Results: Clinically, most patients with these lymphomas present at age 60 years and below, exhibit B symptoms, with elevated serum lactate dehydrogenase (LDH) levels, at least stage III-IV disease at diagnosis, and possess a high International Prognostic Index (IPI) score, collectively indicating a poor prognosis. Conclusion Survival outcomes for patients with DLBCL ranges from three to 37 months. All cases of mortality were associated with DEL, contrasting with DHL cases which had variable outcomes. Due to limited sampling, statistical significance of the results cannot be determined. A comprehensive evaluation is essential to the diagnosis of DLBCL and DHL to include a complete immunohistochemistry panel and molecular testing, notably with FISH studies.
lymphoma ; lymphoma, large B-cell, diffuse ; cytogenetics ; immunohistochemistry

Nephroblastoma is an uncommon renal malignancy primarily observed in the pediatric population, with its occurrence in adults being exceedingly infrequent. We describe an extremely rare case of a malignant embryonal tumor presenting in an adult patient with right renal mass. Final histopathologic diagnosis was nephroblastoma with favorable histology. Use of immunohistochemistry studies is generally unnecessary but its rarity in the adult population raises uncertainty in diagnosing this malignancy by histomorphology alone.

Pemphigus foliaceous is a rare autoimmune blistering disease, while psoriasis is a common immune‑mediated inflammatory skin disease. The coexistence of psoriasis and pemphigus foliaceous has rarely been reported. We report a case of a 55‑year‑old Filipino female with an 8‑year history of chronic plaque‑type psoriasis biopsy‑proven. After 5 years, she developed generalized flaccid bullae and crusted erosions over the face, trunk, and extremities, with no mucous membrane involvement. Skin punch biopsy, direct immunofluorescence, and enzyme‑linked immunosorbent assay were consistent with pemphigus foliaceous. The combination of topical corticosteroids and oral methotrexate was selected as the therapeutic approach, leading to a notable improvement in the patient’s condition. This case report underscores the significance of identifying the simultaneous presence of psoriasis alongside autoimmune blistering diseases like pemphigus foliaceous. Examining predisposing and triggering factors, performing re‑biopsy, and further work‑up as the disease evolves may yield more profound insights. Nonetheless, effectively managing this condition poses a significant challenge.
Fluorescent Antibody Technique, Direct ; Methotrexate ; Psoriasis

Molting is an important physiological phenomenon of many metamorphosis insects, during which the old and new epidermis are separated by enzymes present in the molting fluid. Various proteomic studies have discovered the presence of Bombyx mori carboxypeptidase A (Bm-CPA) in the molting fluid of silkworm, but its function remains unclear. In order to better understand the role of Bm-CPA in the molting process of silkworm, Bm-CPA was analyzed by bioinformatics analysis, real-time fluorescence quantitative PCR, antibody preparation, immunofluorescence staining, and expression in Pichia pastoris. The results showed that Bm-CPA had a conserved M14 zinc carboxypeptidase domain and glycosylation site. Its expression was regulated by ecdysone 20E, and large expression was observed in the epidermis of the upper cluster stage. Immunofluorescence staining showed that Bm-CPA was enriched in the epidermis during the molting stage, and the inhibitor of Bm-CPA led to the larval death due to the inability to molt. We also successfully obtained a large number of recombinant Bm-CPA proteins by Pichia pastoris expression in vitro. These results may facilitate further understanding the molting development process of silkworm.
Animals ; Molting/genetics* ; Bombyx/genetics* ; Carboxypeptidases A/metabolism* ; Proteomics ; Larva/metabolism* ; Fluorescent Antibody Technique ; Insect Proteins/metabolism*