The end of surgical pathology.

The probable future of the specialty of surgical pathology is something that all pathologists would have some interest in. It would be of special interest to educators and young physicians considering entering the specialty. For the purpose of stimulating thought and discussion about this subject, this article proffers opinions about some pertinent factors relevant to the current state-of-the-art and the short- and intermediate-term future.

Giant cell tumor of the larynx: a clinicopathologic series of eight cases and a review of the literature.

True giant cell tumors of the larynx (GCTL) are quite rare, and only individual case reports are documented in the literature. Eight cases of GCTL were identified in the Otorhinolaryngic Pathology Tumor Registry between 1966 and 2000. There were 2 women and 6 men, ages 26 to 62 years (mean, 44.5 yrs). Patients presented with a palpable neck mass (n = 5), airway obstruction (n = 3), hoarseness (n = 3), and dysphagia (n = 2). All tumors involved the thyroid cartilage, a few with local extension. The mean tumor size was 4.1 cm. Histologically, the tumors showed no connection to the surface epithelium and arose in sites of ossification. The tumors had an expansile, infiltrative growth and consisted of numerous multinucleated osteoclast-like giant cells within a cellular stroma composed of plump, oval mononuclear cells. Of interest was that the nuclei of the giant cells were similar to the nuclei of the stromal cells. Treatment included biopsy only with adjuvant therapy (n = 2), local resection (n = 3), and total laryngectomy (n = 3). Follow-up showed 5 patients were alive without evidence of disease (mean follow-up, 6.9 yrs); 2 died of unrelated causes (mean survival, 22.2 yrs). No patients developed recurrences. GCTL are rare tumors that can cause significant airway obstruction. Complete surgical resection yields an excellent outcome without adjuvant therapy.

Sinonasal tract eosinophilic angiocentric fibrosis. A report of three cases.

Eosinophilic angiocentric fibrosis (EAF) is a rare submucosal fibrosis without a well-developed differential diagnosis. Three cases of sinonasal tract EAF were identified in 2 women and 1 man, aged 49, 64, and 28 years, respectively. The patients experienced a nasal cavity mass, maxillary pain, or nasal obstructive symptoms of long duration. The process involved the nasal septum (n = 2), nasal cavity (n = 1), and/or the maxillary sinus (n = 1). There was no evidence for Wegener granulomatosis, Churg-Strauss syndrome, Kimura disease, granuloma faciale, or erythema elevatum diutinum. Histologically, the lesions demonstrated a characteristic perivascular "onion-skin" fibrosis and a full spectrum of inflammatory cells, although eosinophils predominated. Necrosis and foreign body-type giant cells were not identified. Surgical excision was used for all patients, who are all alive but with disease at last follow-up. Sinonasal tract EAF is a unique fibroproliferative disorder that does not seem to have systemic associations with known diseases. The characteristic histomorphologic features permit accurate diagnosis.

Tonsillar lymphangiomatous polyps: a clinicopathologic series of 26 cases.

BACKGROUND: Lymphangiomatous polyps are uncommon benign tumors of the tonsils. METHODS: Twenty-six cases of lymphangiomatous polyps diagnosed between 1980 and 1999 were retrieved from the files of the Otorhinolaryngic-Head and Neck Tumor Registry of the Armed Forces Institute of Pathology. Hematoxylin and eosin-stained slides were reviewed to characterize the histologic features of these tumors. Immunohistochemical stains were performed on 15 cases. Clinical follow-up data were obtained. RESULTS: The patients included 13 males and 13 females, ages 3 to 63 years (mean, 25.2 years). Patients experienced dysphagia, sore throat, and the sensation of a mass in the throat. Symptoms were present from a few weeks to years. The tonsillar masses were unilateral in all cases. Clinically, the lesions were frequently mistaken for a neoplasm (n = 18 patients). Grossly, all of the lesions were polypoid and measured 0.5 to 3.8 cm (mean, 1.6 cm). Histologically, the polyps were covered by squamous epithelium showing variable epithelial hyperplasia, dyskeratosis, and lymphocytic epitheliotropism. The masses showed a characteristic submucosal proliferation of small to medium-sized, endothelial-lined, lymph-vascular channels lacking features of malignancy. Collagen, smooth muscle, and adipose tissue were present in the stroma. Intravascular proteinaceous fluid and lymphocytes were noted. Immunohistochemical findings confirmed the endothelial origin of the vascular proliferation and a mixed lymphoid population. The differential diagnosis included fibroepithelial polyp, lymphangioma, juvenile angiofibroma, and squamous papilloma. In all patients with follow-up, complete surgical excision was curative (mean follow-up, 5.4 years; range, 1 mo to 14 years). CONCLUSIONS: We detail the clinical and pathologic features of tonsillar lymphangiomatous polyps. These tumors are uncommon and may clinically be mistaken for a malignant neoplasm. The characteristic histologic features should allow for its correct diagnosis and differentiation from similar appearing tonsillar lesions.

Clarity on the diagnosis line (the devil is in the details)

The diagnosis line of a surgical pathology report obviously influences therapy and is often critical for proper initiation of same. If the word choice, phrasing, or terminology in the diagnosis is potentially ambiguous or subject to misinterpretation, this could have adverse or sometimes even disastrous consequences. Usually the potential for misinterpretation is a subtle facet of the wording and is not apparent to the pathologist. This discussion aims to help the pathologist become more aware of the types of subtle wording nuances that can be important and we hope to foster the habit of searching for and correcting such potentially troublesome nuances.

Exophytic and papillary squamous cell carcinomas of the larynx: A clinicopathologic series of 104 cases.

Exophytic and papillary squamous cell carcinomas (SCCs) are uncommon variants of SCC of the upper aerodigestive tract mucosa. The histomorphologic distinction between these variants has not been previously attempted or correlated with prognostic outcome. One hundred four cases of exophytic and papillary SCCs of the larynx were identified in the files of the Armed Forces Institute of Pathology from 1971 to 1991. The patients included 25 women and 79 men, aged 27 to 89 years (average 60.7 years). Patients had hoarseness at presentation, and many patients were using tobacco (n = 87) and/or alcohol (n = 49). Tumors measured up to 6 cm in greatest dimension. The larger tumors were associated with vocal cord impairment (n = 39). Histologically, the SCCs were divided into 2 growth patterns: papillary-frond (n = 12) or broad-based, exophytic (n = 92). Patients were treated with excisional biopsy, vocal cord stripping, and/or laryngectomy, in conjunction with radiation therapy (n = 70). Eighty-seven patients had no evidence of disease at last follow-up (average follow-up 8.6 years). Seventeen patients with an exophytic pattern died with disease (10 disseminated disease; 7 local disease). No patients with papillary patterns died of disease, although there had been 4 recurrences. In conclusion, patients with papillary and exophytic SCCs have a better prognosis than those with conventional SCCs, and the prognosis for those with papillary SCCs is even better.

Atypical chondroma of the cricoid cartilage: fine-needle aspiration cytologic and histopathologic findings.

Chondroma of the laryngeal skeleton is a rare, benign neoplasm manifested either as a neck mass or, if situated within the airway, as slowly progressive obstruction, hoarseness, or dyspnea. The most common site is the posterior plate of the cricoid cartilage. An atypical perichondrial chondroma arising from the anterior plate of the cricoid cartilage membrane as a neck mass in a young female is presented. The diagnosis was made on a fine-needle aspiration of the mass and subsequently confirmed by histologic examination of the excised mass. Although rare, cartilaginous tumors of the laryngeal skeleton can manifest as a neck mass, and the diagnosis can be made by fine-needle aspiration biopsy in combination with radiographic and clinical examinations. Therefore, the existence and inclusion of these tumors in the differential diagnosis of neck masses by aspiration biopsy should be considered by clinicians and pathologists, and especially cytopathologists, when cartilaginous components are encountered.

The clinical importance of cystic squamous cell carcinomas in the neck: a study of 136 cases.

BACKGROUND: Predominantly cystic squamous cell carcinomas in the neck often present without a clinically apparent primary and therefore are frequently considered to be of branchial cleft origin. It is the authors' hypothesis that the anatomic site of the primary carcinoma that produced the neck metastasis can often be predicted on the basis of the histologic features. METHODS: Cases of cystic squamous cell carcinoma in the neck diagnosed between 1971 and 1991 were retrieved from the Otorhinolaryngic Pathology Registry of the Armed Forces Institute of Pathology. Histologic features were reviewed and patient follow-up was obtained and analyzed. RESULTS: In cases wherein the primary site was discovered subsequently, 64% of the primaries were in the lingual or faucial tonsil. An additional 8% of cases were in nasopharyngeal tonsillar tissue. The cases that did not originate in Waldeyer's tonsillar ring generally differed in histologic appearance from the tonsillar cases. The tonsillar primaries were discovered within an average of 12.4 months, but many were not discovered for years (up to 11 years). Most were small, indicating a slower growth of the primary than is usually expected for squamous cell carcinoma. Patients with such carcinomas had a much better prognosis than patients with metastatic squamous cell carcinomas of other upper airway mucosal sites. CONCLUSIONS: In most cases of prominently cystic squamous cell carcinomas in the upper neck, the origin of the primary site will be in faucial or lingual tonsillar crypt epithelium. Knowledge of the probable site of origin allows for more tailored therapy in which the patients can be treated relatively conservatively with surgical excision and subsequent field-limited radiation therapy only, with 77% survival at 5 years. None of the cases reviewed in this study was a branchiogenic carcinoma.

Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease.

OBJECTIVES: Isolated reports suggest a possible association of endolymphatic sac tumors (ELSTs), which are extremely rare in the general population, with von Hippel-Lindau disease (VHL). To determine if hearing loss and ELSTs are a component of VHL, we examined prevalence, clinical presentation, and natural history of hearing loss and ELSTs in VHL. DESIGN: Brain magnetic resonance images (MRIs) from 374 patients screened for VHL were reviewed for evidence of ELSTs. The VHL patients with MRI evidence suggestive of ELSTs or a history of hearing loss, tinnitus, or vertigo underwent additional radiologic and audiologic evaluations. To further assess prevalence of hearing loss and ELST in VHL, the next 66 patients screened in the VHL clinic (49 with proven VHL, 17 at risk for VHL) received MRI and audiologic assessment. SETTING: Referral center. PARTICIPANTS: Study subjects comprised 374 persons screened for VHL, 66 consecutive patients with VHL or at risk for VHL, 4 patients with 6 ELSTs, and 13 previously reported patients with VHL and invasive tumors of the temporal bone. INTERVENTION: Magnetic resonance image and computed tomographic (CT) scan of the posterior fossa and audiologic assessment. MAIN OUTCOME MEASURES: Any ELST visible on MRI or CT and hearing loss compatible with ELST. RESULTS: Magnetic resonance imaging revealed evidence of 15 ELSTs in 13 (11%) of 121 patients with VHL, but in none of the 253 patients without evidence of VHL (P<.001). Clinical findings in these 13 patients included hearing loss (13), tinnitus (12), vertigo (8), and facial paresis (1). Mean age at onset of hearing loss was 22 years (range, 12-50 years). Hearing for pure tones was abnormal in all affected ears and in 6 of the 11 additional, allegedly unaffected ears. In 8 patients (62%), hearing loss was the first manifestation of VHL. Presence or absence of hearing loss was associated with duration of symptoms (P<.002) and with tumor size (P<.01). Further, 43 (65%) of the 66 patients from the VHL clinic had pure tone threshold abnormalities, abnormalities that occurred bilaterally in 23 (54%) of the 43 affected subjects; however, evidence is lacking for a definitive association with ELST (3 [6%] of 49 patients with proven VHL had ELST evident on MRI). CONCLUSIONS: Hearing loss and ELSTs are frequently associated with VHL syndrome and should be considered when screening individuals at risk for VHL and when monitoring patients with an established diagnosis of VHL. Many patients with VHL have hearing loss without radiographic evidence of an ELST. Whether it is caused by an ELST that is too small to be detected by MRI or is produced by some other etiology is still unknown. Audiologic evaluation and MRI should allow early detection and enhance management of hearing loss in these patients.

Botryomycosis ('bacterial ball') of the sinonasal tract caused by Pseudomonas aeruginosa.

BACKGROUND: Botryomycosis is a chronic bacterial infection that typically presents as a cutaneous lesion. Visceral involvement may occur, but mucosal disease is uncommon. We report two cases of sinonasal tract botryomycosis that clinically simulated a neoplasm. METHODS: Two cases of sinonasal tract botryomycosis were identified from the Otolaryngic Tumor Registry at the Armed Forces Institute of Pathology, Washington, DC. The clinical records, slides, and paraffin blocks were available for both cases. Histochemical stains, including Brown and Hopps, Gomori's methenamine-silver, acid-fast bacilli, mucicarmine, periodic acid-Schiff, and Warthin-Starry, were performed. RESULTS: The patients were an 81-year-old man and a 43-year-old woman. The man presented with acute ethmoiditis and a bulging eye. Radiographic studies showed a soft tissue mass in his left maxillary antrum with osseous erosion of adjacent anatomic sites. The woman presented with persistent headaches of more than 1 year's duration, with increasing severity in the months prior to presentation. An expansile soft tissue mass was identified in her right maxillary and ethmoid sinuses. Surgery was performed on both patients. The histology included amorphous, acellular material and separate, rounded eosinophilic granules associated with a neutrophilic infiltrate. A Splendore-Hoeppli phenomenon was seen. Filamentous gram-negative bacilli, identifiable only by histochemical staining, were morphologically compatible with Pseudomonas aeruginosa. Cultures of samples taken from both patients intraoperatively confirmed the organisms as P. aeruginosa. CONCLUSIONS: Sinonasal botryomycosis is a rare localized disease that may be mistaken clinically for an aggressive neoplasm. Complete surgical evacuation is curative.

Pharyngeal dermoids ("hairy polyps") as accessory auricles.

The purpose of this study is to clarify the origin and nature of so-called hairy polyps or dermoids of the pharynx, which are often thought to be a variant of pharyngeal teratoma. For this purpose, a case is reported of a dermoid polyp involving the middle ear of an infant, the features of multiple examples of pharyngeal dermoid polyps and teratomas received for consultation by the Armed Forces Institute of Pathology are examined, and selected pertinent reports from the literature are reviewed. All three means are used to support the conclusion that these lesions are choristomatous developmental anomalies arising from the first branchial cleft area and that they essentially represent heterotopic accessory "ears" (auricles) without the growth potential of a teratoma.

Lymphoid changes of the nasopharyngeal and palatine tonsils that are indicative of human immunodeficiency virus infection. A clinicopathologic study of 12 cases.

We report 12 cases in which the histomorphologic changes of the nasopharyngeal tonsils (adenoids) or palatine tonsils suggest infection with the human immunodeficiency virus (HIV). The patients included 10 men and two women, aged 20 to 42 years (median, 33 years). The clinical presentation included airway obstruction, pharyngitis, fever, and a tonsillar or adenoidal mass lesion. Histologic evaluation of the excised adenoids or tonsils in 10 of the cases demonstrated a spectrum of changes including florid follicular hyperplasia, follicle lysis, attenuated mantle zone, and the presence of multinucleated giant cells (MGC). The latter characteristically localized adjacent to the surface or tonsillar crypt epithelium. Two of the 12 cases showed marked lymphoid depletion with absent germinal centers, plasmacytosis, and stromal vascular proliferation. Immunohistochemical evaluation for HIV p24 core protein showed reactivity in 10 of 12 cases localized to follicular dendritic cell network (FDC), the MGC, scattered interfollicular lymphoid cells, and cells identified within the surface or crypt epithelium. Localization of viral RNA by in situ hybridization paralleled the HIV p24 immunohistochemical findings. Additional significant findings included the presence of both CD-68 and S-100 protein in the MGC and the presence of S-100 protein in dendritic cells. Other than HIV, no microorganisms were identified. At the time of presentation, eight patients were not known to be a risk for HIV infection, nor were they known to be HIV infected or suffering from AIDS. In these patients, HIV infection was suspected on the basis of the histologic changes seen in the resected tonsillar and adenoidal tissue. Serologic evaluation (by enzyme-linked immunosorbent assay), confirmed the presence of HIV infection. Our findings suggest the possibility of HIV dissemination through the upper aero-digestive tract mucosa via target cells, such as intraepithelial dendritic cells, submucosal macrophages, and T-lymphocytes. Subsequent presentation of viral antigens to the tonsillar and adenoidal lymphoid tissues results in enlargement of these structures that clinically may simulate a neoplastic proliferation but causes histomorphologic changes that are highly suspicious for HIV infection even in asymptomatic HIV-positive patients.

Aggressive psammomatoid ossifying fibromas of the sinonasal region: a clinicopathologic study of a distinct group of fibro-osseous lesions.

BACKGROUND: Psammomatoid ossifying fibromas represent a unique subset of fibro-osseous lesions of the sinonasal tract. They have distinctive histomorphologic features and a tendency toward locally aggressive behavior, including invasion and destruction of adjacent anatomic structures. METHODS: Seven cases of psammomatoid ossifying fibromas of the sinonasal tract were identified in the files of the Otolaryngic Tumor Registry at the Armed Forces Institute of Pathology. Medical records, including the clinical history, location of the lesions, radiographs, treatment, and follow-up were reviewed in each case. Follow-up information was available in all of the cases. RESULTS: Four of the patients were male and three were female. The patient's ages ranged from 5 to 54 years (median age, 33 years). Symptoms included facial swelling, nasal obstruction, pain, sinusitis, headache, and proptosis. Radiographic studies confirmed the presence of an osseous and/or soft tissue mass varying in appearance from well demarcated without invasion or erosion to invasive with bone erosion and intracranial extension. Sites of involvement included the nasal cavity and all paranasal sinuses, particularly the ethmoid and maxillary sinuses. Often, more than one sinus was involved and extension of disease included involvement of the orbit, nasopharynx, palate, and anterior cranial fossa. The histologic appearance was characterized by the presence of small mineralized (psammomatoid) bodies admixed with a cellular stroma with a variable amount of myxomatous material and scattered giant cells. Confusion with other osseous and soft tissue tumors may occur resulting in too limited or too aggressive management. En bloc surgical excision is the treatment of choice and may prove curative. Aggressive behavior with recurrence(s) or invasion into adjacent structures occurred. At the time of this writing, the patients are alive over follow-up periods ranging from 6 months to 7 years. CONCLUSIONS: Gnathic and midfacial fibro-osseous proliferations are a diverse group of lesions. A subset of these fibro-osseous lesions with predilection for the sinonasal tract were identified. These lesions are characterized by their distinctive histology, including psammomatoid ossicles and their locally aggressive growth. Complete surgical removal is the treatment of choice.

Respiratory epithelial adenomatoid hamartomas of the sinonasal tract and nasopharynx: a clinicopathologic study of 31 cases.

We report the clinicopathologic features of 31 cases of respiratory epithelial adenomatoid hamartomas occurring in the nasal cavity, paranasal sinuses, and nasopharynx. The patients included 27 men and 4 women ranging in age from 27 to 81 years (median, 58 years). Symptoms included nasal obstruction, nasal stuffiness, deviated septum, epistaxis, and chronic (recurrent) rhinosinusitis. The symptoms occurred over various time periods from as short as a few months to up to 8 years in duration. Physical examination identified the presence of a polypoid mass lesion(s), most often identified in one or both nasal cavities (n = 22). Within the nasal cavity the most common site of occurrence was the nasal septum, particularly along its posterior aspect. Other areas within the nasal cavity were also involved, as were the ethmoid sinus, frontal sinus, and nasopharynx. The gross appearance of the mass lesions suggested a diagnosis of an inflammatory polyp, but because of subtle differences, including frequent occurrence along the nasal septum and a more indurated quality, these polyps were considered unusual for the typical inflammatory polyps. Histologically, these lesions were characterized by a prominent glandular proliferation lined by ciliated respiratory epithelium originating from the surface epithelium. The differential diagnosis of these adenomatoid hamartomas includes schneiderian papillomas of the inverted type and adenocarcinomas. Diagnostic misinterpretations may result in untoward surgical intervention. Limited but complete surgical resection was the treatment of choice, following which there were no instances of recurrent disease.

Liposarcomas of the larynx and hypopharynx: a clinicopathologic study of eight new cases and a review of the literature.

Laryngeal and hypopharyngeal liposarcomas were studied in seven men and one woman. Patient age ranged from 25 to 81 years, with a median of 64 years. Symptoms included dysphagia, airway obstruction, and the sensation of a foreign body in the back of the throat. Histologically, seven of the tumors were of the biologically favorable types, either representing well-differentiated (lipoma-like) liposarcomas or myxoid liposarcomas. One tumor was a pleomorphic liposarcoma. Six of the eight patients had one or more episodes of recurrent tumor. Surgery is the treatment of choice and can include conservative (organ-sparing) procedures. However, to eradicate the tumor completely and thereby prevent recurrent disease, open surgical approaches (i.e., lateral pharyngotomy), rather than endoscopic techniques should be employed. The morbidity rate for laryngeal and hypopharyngeal liposarcomas is high because these tumors tend to recur over extended periods of time. In this study, however, there were no instances of metastatic disease, and no deaths were attributed to liposarcoma. Prospectively, laryngeal and hypopharyngeal well-differentiated (lipoma-like) liposarcoma is a difficult clinical and histopathologic diagnosis to establish. Often, this diagnosis is made only after one or more episodes of recurrent disease.

Atypical stromal cells in inflammatory nasal polyps: immunohistochemical and ultrastructural analysis in defining histogenesis.

The authors investigated 29 cases of sinonasal polyps with atypical stromal cells (ASC). The clinicopathologic features of these lesions were of benign inflammatory polyps except for the presence of ASC. Misinterpretation of these cells resulted in contributor diagnosis of sarcoma (rhabdomyosarcoma). Immunohistochemical study of the ASC demonstrated the presence of actin (smooth muscle and muscle specific), KP-1, and vimentin; no reactivity was seen with desmin, myoglobin, S-100 protein, or glial fibrillary acidic protein (GFAP). Unexpectedly, cytokeratin reactivity was identified in more than 75% of the cases analyzed. Ultrastructural analysis revealed that the ASC shared morphologic features in common with fibroblasts and smooth muscle cells. Based on the light microscopic, immunohistochemical, and ultrastructural findings, it was concluded that the ASC represent reactive myofibroblasts and not a neoplastic proliferation. Follow-up data supported this contention indicating the absence of an aggressive biological course. Misinterpretation as a malignant neoplasm might result in unwarranted and unnecessary therapeutic intervention.

Epidermoid cyst (cholesteatoma) and cholesterol granuloma of the temporal bone and epidermoid cysts affecting the brain.

Five cases of petrous apex epidermoid cysts, six cases of petrous apex cholesterol granuloma cysts, and seven cases of intradural cerebellopontine angle (CPA) or intracranial epidermoid cysts are reviewed. Petrous epidermoid cysts (cholesteatomas) and cholesterol granuloma cysts cannot be differentiated definitely from each other on CT scans. On MR imaging, epidermoid cysts exhibit long T1 and long T2 characteristics and can be differentiated from cholesterol granuloma cysts, which exhibit short T1 and long T2 characteristics. Intradural epidermoid cysts involving the CPA or other portions of the brain, also demonstrate long T1 and long T2 characteristics on MR scans. Intradural epidermoid cysts exhibit curvilinear areas of higher intensity on T1-weighted images, which were best evaluated on T1-weighted MR images obtained with more averages (6 to 8).