An unusual initial presentation of mantle cell lymphoma arising from the lymphoid stroma of warthin tumor.

BackgroundWarthin tumors presenting concomitantly with a lymphoma is vanishingly rare with only 15 reported cases in English literature. Herein, we report an unusual initial presentation of a mantle cell lymphoma involving the lymphoid stroma of a Warthin tumor.Case presentationA seventy-seven year old otherwise healthy gentleman with a 50-pack year smoking history presents with a slowly enlarging left cheek mass. CT scan of the neck demonstrated a left parotid gland tumor measuring 3.4 cm in greatest dimension. He underwent a left superficial parotidectomy, with subsequent histopathologic examination revealing a Warthin tumor with extensive expansion of the lymphoid stroma. Flow cytometric, immunohistochemical, and cytogenetic studies of the stromal component of the tumor confirmed the presence of a mantle cell lymphoma. Clinical staging demonstrated stage IVa disease, and was considered to be at low to intermediate risk due to the slow growth of the parotid lesion. The patient is undergoing close follow up with repeat PET-CT scans at six months.ConclusionTo the best of our knowledge, this is the first well documented collision tumor between mantle cell lymphoma and a Warthin tumor. This case also brings to light the significance of thorough evaluation of the lymphoid component of Warthin tumor

Incidental finding of lymphoma after septoplasty.

IntroductionSeptoplasty, or surgical correction of the deviated septum, is an elective, routinely performed rhinologic procedure to address nasal airway obstruction. In many cases, resected septal cartilage and bone fragments are sent for pathologic review, although there is no consensus on this practice. We reported two cases of incidentally diagnosed lymphoma after elective septoplasty and discussed clinical presentation, diagnosis, and management.MethodsRetrospective chart review of two patients who underwent septoplasty at a tertiary academic medical center and found to have incidental lymphoma based on histopathology.ResultsTwo patients who underwent septoplasty had an incidental diagnosis of lymphoma on pathologic analysis. One patient was noted to have an S-shaped septal deviation that produced bilateral nasal obstruction. She underwent a difficult septoplasty, in which the mucoperichondrial flap was firmly adherent to the underlying septum and bone. Final pathology demonstrated diffuse large B-cell lymphoma. She was treated with chemoradiation and remained free of disease at 59 months. The other patient had a history of nasal trauma, which produced left septal deviation. He underwent an uncomplicated septoplasty, with pathology that demonstrated low-grade B-cell lymphoma. Because there was no evidence of active disease, the decision was made to not treat and to observe the patient clinically.ConclusionsThis is the first reported series of septal lymphoma incidentally diagnosed on routine septoplasty. Although histopathologic review of specimens from routine nasal and sinus surgery is not routinely performed, this report highlighted the importance of this process, on a case-by-case basis, in detecting unexpected malignancies that otherwise were clinically silent

Quantitative Alterations in Cutaneous Langerhans Cells During the Evolution of Malignant Melanoma of the Skin

Melanomas are associated with a T-cell predominant infiltrate that may cause their regression. Langerhans cells (LC) are essential for initiation and maintenance of specific T-cell- mediated responses in the skin. Therefore, a change in this antigen-presenting LC population may alter the host response. To determine whether the LC population varies during the evolution of primary cutaneous melanoma 32 melanocytic lesions, nevi, and cutaneous melanomas were studied by quantitative immunohistology. The monoclonal antibody, Leu-6, and the avidin biotin complex immunoperoxidase method were used to identify LC. Compared with histologically normal melanoma-adjacent skin, epidermal LC were depleted above “deeply invasive” melanomas but were relatively unchanged above nevi, “early invasive” melanomas, and cutaneous metastatic melanoma nodules. Dermal LC were significantly increased around in situ and “early invasive” melanomas but not around “deeply invasive” melanomas or cutaneous metastatic nodules. Dermal LC are thus associated with early transformed melanocytes and may present neoantigens to T lymphocytes in situ or after LC maturation in the draining lymph node. Melanoma-associated LC decline in number as melanoma progresses

An unusual initial presentation of mantle cell lymphoma arising from the lymphoid stroma of warthin tumor

BACKGROUND: Warthin tumors presenting concomitantly with a lymphoma is vanishingly rare with only 15 reported cases in English literature. Herein, we report an unusual initial presentation of a mantle cell lymphoma involving the lymphoid stroma of a Warthin tumor. CASE PRESENTATION: A seventy-seven year old otherwise healthy gentleman with a 50-pack year smoking history presents with a slowly enlarging left cheek mass. CT scan of the neck demonstrated a left parotid gland tumor measuring 3.4 cm in greatest dimension. He underwent a left superficial parotidectomy, with subsequent histopathologic examination revealing a Warthin tumor with extensive expansion of the lymphoid stroma. Flow cytometric, immunohistochemical, and cytogenetic studies of the stromal component of the tumor confirmed the presence of a mantle cell lymphoma. Clinical staging demonstrated stage IVa disease, and was considered to be at low to intermediate risk due to the slow growth of the parotid lesion. The patient is undergoing close follow up with repeat PET-CT scans at six months. CONCLUSION: To the best of our knowledge, this is the first well documented collision tumor between mantle cell lymphoma and a Warthin tumor. This case also brings to light the significance of thorough evaluation of the lymphoid component of Warthin tumor

Temporal bone verrucous carcinoma: outcomes and treatment controversy

Verrucous carcinoma is a rare tumor that presents in the head and neck with the most common sites being the oral cavity and larynx. Fourteen cases of verrucous carcinoma of the temporal bone have been described in literature; this study aims to examine treatment outcomes and discuss the controversy surrounding postoperative radiation. The study design included a literature review along with individual case report in the setting of a tertiary care medical center. Outcome analysis of all cases of verrucous carcinoma of the temporal bone, which are documented in the English literature, and presentation of a single patient report including gross, histologic and radiologic analyses were performed. The longest recorded survival for verrucous carcinoma of the temporal bone occurs in patients treated with surgery alone. Poorer outcomes for patients treated with adjuvant (chemo)radiation may be due to more advanced stage of disease at the time of treatment. Early reports of radiation leading to tumor dedifferentiation or early recurrence are not supported by more recent studies. Whether adjuvant radiation therapy is indicated in verrucous carcinoma of the temporal bone remains controversial

Schneiderian papillomas: comparative review of exophytic, oncocytic, and inverted types.

BackgroundSinonasal papillomas are benign epithelial neoplasms arising from Schneiderian mucosa. The three subtypes, exophytic, oncocytic, and inverted (inverted papilloma [IP]), should be distinguished from one another histopathologically. This study (1) highlights the histopathological and clinical differences between the Schneiderian papilloma subtypes and (2) identifies clinical features that potentially predict papilloma subtypes.MethodsA retrospective review was performed of patients with Schneiderian papillomas over an 11-year period.ResultsSeventy patients with sinonasal papillomas who underwent sinus surgery were identified. There were 50 (71%) male and 20 (29%) female subjects diagnosed at an average age of 53 years (range, 13-80 years). Exophytic (n = 25), oncocytic (n = 9), and IP (n = 37) were identified. IP was associated with transformation into squamous cell carcinoma in three (8%) cases and dysplasia in three (8%) cases. Neither oncocytic nor exophytic subtypes were associated with dysplasia or malignancy. On multivariate analysis of potential predictors of papilloma subtype, history of chronic rhinosinusitis (CRS) and location of papilloma were significantly associated with papilloma subtype. Using classification and regression tree model, papilloma subtypes can be predicted based on presence or absence of CRS and papilloma location with nominal 82.4% accuracy.ConclusionThe inverted and exophytic type are the most common sinonasal papillomas, with the inverted type having an 8% rate of malignant transformation in this study. In contrast, the oncocytic type was not associated with dysplasia or malignancy in our series despite reports in the literature indicating malignant potential. History of CRS and papilloma location can provide clues to the histological subtype, which is important for surgical planning and patient counseling

Schneiderian papillomas: comparative review of exophytic, oncocytic, and inverted types.

BackgroundSinonasal papillomas are benign epithelial neoplasms arising from Schneiderian mucosa. The three subtypes, exophytic, oncocytic, and inverted (inverted papilloma [IP]), should be distinguished from one another histopathologically. This study (1) highlights the histopathological and clinical differences between the Schneiderian papilloma subtypes and (2) identifies clinical features that potentially predict papilloma subtypes.MethodsA retrospective review was performed of patients with Schneiderian papillomas over an 11-year period.ResultsSeventy patients with sinonasal papillomas who underwent sinus surgery were identified. There were 50 (71%) male and 20 (29%) female subjects diagnosed at an average age of 53 years (range, 13-80 years). Exophytic (n = 25), oncocytic (n = 9), and IP (n = 37) were identified. IP was associated with transformation into squamous cell carcinoma in three (8%) cases and dysplasia in three (8%) cases. Neither oncocytic nor exophytic subtypes were associated with dysplasia or malignancy. On multivariate analysis of potential predictors of papilloma subtype, history of chronic rhinosinusitis (CRS) and location of papilloma were significantly associated with papilloma subtype. Using classification and regression tree model, papilloma subtypes can be predicted based on presence or absence of CRS and papilloma location with nominal 82.4% accuracy.ConclusionThe inverted and exophytic type are the most common sinonasal papillomas, with the inverted type having an 8% rate of malignant transformation in this study. In contrast, the oncocytic type was not associated with dysplasia or malignancy in our series despite reports in the literature indicating malignant potential. History of CRS and papilloma location can provide clues to the histological subtype, which is important for surgical planning and patient counseling

Hepatic Fibrinogen Storage Disease in a Patient with Hypofibrinogenemia: Report of a Case with a Missense Mutation of the FGA Gene.

We report a 9-year-old patient with abnormal liver tests found incidentally during routine bloodwork as part of a preoperative evaluation for excision of a benign cyst. A liver biopsy demonstrated hepatocytes to have pale and expanded cytoplasm that contained multiple vague globular eosinophilic inclusions. Electron microscopy showed fingerprint-like structures in the dilated cisternae of the rough endoplasmic reticulum, characteristic of fibrinogen. Whole exome sequencing identified a heterozygous missense mutation at codon 35 of the fibrinogen α (FGA) gene. No mutation was identified in the β or γ chains. His plasma fibrinogen levels were found to be decreased to 85 mg/dL (normal range 215-464). His family history was pertinent for his mother and maternal grandfather with hypofibrinogenemia. He had not had any significant bleeding episodes except for minor bruising over the shins. This case illustrates a rare etiology of storage disease that causes abnormal liver function tests

Histopathological and ultrastructural analysis of vestibular endorgans in Meniere's disease reveals basement membrane pathology.

BackgroundWe report the systematic analysis of the ultrastructural and cytological histopathology of vestibular endorgans acquired from labyrinthectomy in Meniere's disease.Methods17 subjects with intractable Meniere's disease and ipsilateral non-serviceable hearing presenting to the Neurotology Clinic from 1997 to 2006 who chose ablative labyrinthectomy (average age = 62 years; range 29-83 years) participated. The average duration of symptoms prior to surgery was 7 years (range 1-20 years).ResultsNearly all vestibular endorgans demonstrated varying degrees of degeneration. A monolayer of epithelial cells occurred significantly more frequently in the horizontal cristae (12/13 = 92%) (p < 0.001), the superior cristae (5/5 = 100%) (p < 0.005), the posterior cristae (2/2) compared with the utricular maculae (4/17 = 24%). Basement membrane (BM) thickening was more common in all of the cristae ampullares (18 out of 20) than the utricular maculae. Although only four saccular maculae were obtained, 3 out of 4 exhibited BM thickening and monolayer degeneration. Monolayer degeneration was highly significantly correlated with the presence of BM thickening (p < 0.001). Other degenerative changes noted equally among the five vestibular endorgans which were not significantly correlated with BM thickening or monolayer degeneration included hair cell vacuolization and stereocilia loss, microvesicles in the supporting cells, and increased stromal intercellular spaces. Transmission electron microscopy demonstrated disorganization of the BM collagen-like fibrils, and normal ultrastructural morphology of the nerve terminals and myelinated fibers. Stromal fibroblasts and endothelial cells of stromal blood vessels demonstrated vacuolization, and stromal perivascular BMs were also thickened.ConclusionSystematic histopathological analysis of the vestibular endorgans from Meniere's disease demonstrated neuroepithelial degeneration which was highly correlated with an associated BM thickening. Other findings included hair cell and supporting cell microvessicles, increased intercellular clear spaces in the stroma, and endothelial cell vacuolization and stromal perivascular BM thickening