Carcinoma

Meningeal carcinomatosis in undifferentiated nasopharyngeal carcinoma: A case report

July 13, 2014     Daniel M. Cushman, MD; German Giese, MD; Panta Rouhani, MD, PhD, MPH
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Abstract

Meningeal carcinomatosis is the tumoral invasion of the leptomeninges. It is caused by the spread of malignant cells throughout the subarachnoid space, which produces signs and symptoms due to multifocal involvement. Cranial nerve symptoms are the most common focal findings. The diagnosis is usually made by imaging and/or cytology. Head and neck cancers are the cause of approximately 2% of all cases of meningeal carcinomatosis; in very rare cases, they are caused by a nasopharyngeal carcinoma. We report a case of meningeal carcinomatosis that was caused by a recurrence of undifferentiated nasopharyngeal carcinoma. The patient, a 60-year-old woman, experienced no focal neurologic symptoms and exhibited no radiologic evidence of meningeal involvement. We also review the literature on meningeal carcinomatosis secondary to nasopharyngeal carcinoma.

Metastatic hepatocellular carcinoma presenting as a sphenoid sinus mass and meningeal carcinomatosis

June 8, 2014     Hilwati Hashim, MBBCh, MRad; Kartini Rahmat, MBBS, MRad; Yang Faridah Abdul Aziz, MBBS, MRad; Patricia Ann Chandran, MD, MPath
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Abstract

We report the case of a 30-year-old woman who was referred to us for evaluation of a 2-week history of fever, headache, vomiting, bilateral ptosis, and blurred vision. Imaging obtained by the referring institution had identified a sphenoid sinus mass and diffuse meningeal infiltration, which was thought to represent an infective process. We subsequently identified the mass as a metastatic hepatocellular carcinoma (HCC). The patient was placed under palliative care, and she died 1 month later. Metastases to the sphenoid sinus from any primary source are very rare, and they are generally not considered in the radiologic differential diagnosis. HCC is known to metastasize to the lung, lymph nodes, and musculoskeletal system; again, reported cases of metastasis to the sphenoid sinus are rare. Indeed, our review of the English-language literature found only 6 previously reported cases of sinonasal metastasis of a primary HCC. A diagnosis of a sinonasal metastasis is more difficult in a patient who has no previous diagnosis of a primary malignancy. In presenting this case, our aim is to remind readers of this possibility.

Sebaceous carcinoma of the parotid gland: A case report and review of the literature

June 8, 2014     Alexander Manteghi, DO; Seth Zwillenberg, MD; Vivian Arguello-Guerra, MD
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Abstract

Sebaceous carcinoma is a rare malignancy primarily affecting the periocular sebaceous glands. Sebaceous carcinoma of the parotid gland is exceedingly rare, with only 30 cases reported in the literature. Our case brings this total to 31. We present a case involving a 57-year-old man with a slowly enlarging, right tail-of-parotid mass abutting the ear lobule with overlying skin discoloration. Excision of the mass with superficial parotidectomy was performed, and microscopic examination demonstrated sebaceous carcinoma with positive margins. Subsequently, the patient underwent a completion parotidectomy and inferior auriculectomy with no evidence of residual tumor. Six months after postoperative adjuvant radiotherapy, the patient remains tumor-free. The clinical and morphologic features of this tumor are discussed.

Extensive basal cell carcinoma of the face: An extreme case of denial

June 8, 2014     Soroush Zaghi, MD; Pedram Ghasri, MD; Paul Busse, MD, PhD; John Clark, MD; Kevin Emerick, MD
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Abstract

Patients with head and neck cancer are particularly susceptible to using denial as a coping mechanism. While some forms of denial may help patients achieve better levels of physical functioning, persistent denial can serve as a major barrier to treatment. We report a case of extreme denial by a 60-year-old woman with an extensive basal cell carcinoma of the face that had been neglected for more than 20 years. We present this case to raise awareness of the potential danger of denial, and we discuss strategies that physicians can undertake to properly manage patients who engage in it. Since the diagnosis and treatment of head and neck cancer can result in profound psychological trauma, gaining an appreciation for how patients cope with it is an important part of the comprehensive care of head and neck oncology patients.

Metastatic cervical carcinoma from an unknown primary: Literature review

May 7, 2014     Rodrigo Arrangoiz, MS, MD; Tom J. Galloway, MD; Pavlos Papavasiliou, MD; John A. Ridge, MD, PhD; Miriam N. Lango, MD
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Abstract

Carcinoma of an unknown primary (CUP) encompasses a heterogeneous group of tumors for which no primary site can be detected following a thorough history, physical examination, and noninvasive and invasive testing. CUP presenting with metastasis to the neck (metastatic cervical carcinoma from an unknown primary [MCCUP]) has been an enigma since von Volkmann first described it in 1882 as a cancer arising in a branchial cleft cyst. Genetic studies have shed some light on this unusual entity. In most cases, clinical features, imaging studies, and a meticulous assessment of the upper aerodigestive tract should assist in identifying the source of disease. Molecular testing of cytologic specimens for Epstein-Barr virus and human papillomavirus (HPV) can facilitate identification of the primary site in the nasopharynx and oropharynx. At least 25% of MCCUPs are directly attributable to HPV-related malignancies, and this number can be expected to increase. Minimally invasive transoral mucosal sampling can identify an otherwise clinically and radiologically occult cancer. We performed a literature review with the objective of discussing the history, epidemiology, clinical presentation, diagnostic workup, and management of MCCUP.

Early detection of nasopharyngeal carcinoma using IgA anti-EBNA1 + VCA-p18 serology assay

March 18, 2014     Achmad C. Romdhoni, MD, PhD; Nurul Wiqoyah, MS; Widodo Ario Kentjono, MD, PhD
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Nasopharyngeal carcinoma (NPC) is the most common head and neck malignancy in Indonesia. Overall, it ranks fourth in males and sixth in females as the most prevalent type of cancer in that country. The data show that in the year 2011, NPC incidence was considered to be intermediate (6.2/100,000 population per year). Through histopathologic examination, about 70 to 80% of these cases were found to be type III according to the WHO classificaton. NPC carries an excellent prognosis if treated early, but most patients presented with stage III to IV disease, which negatively affected the cure rate and increased the mortality rate. Epstein-Barr virus (EBV) IgA serology has been established as an effective marker for NPC. Therefore, biologic markers, DNA, and/or antibody-based diagnosis is needed to decrease NPC cases. A screening program needs to be developed that will identify people at high risk of NPC and those who are in the early stage of the disease. In this study, 20 samples were collected from posttherapy patients. An otolaryngologic examination, histopathology of nasopharyngeal tissue, and blood testing for serologic markers were performed. IgA anti-EBNA1 + VCA-p18 enzyme-linked immunosorbent assay showed positive impact as a tool for confirming the diagnosis of NPC, but it still has to be combined with other specific diagnostic tools for post-therapy monitoring and for determining prognosis.

Merkel cell carcinoma

March 18, 2014     Jeffrey D. Shiffer, MD; Lester D.R. Thompson, MD
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The expected 5-year survival rate for patients with Merkel cell carcinoma is more than 80% if the tumor is less than 2 cm and has not metastasized. Once a tumor has metastasized regionally, the 5-year survival rate drops to about 50%.

Basaloid squamous cell carcinoma of the pinna: Report of a rare case

January 21, 2014     Anil Jain, MS; Ashish Katarkar, MS; Pankaj Shah, MS; Jignasa Bhalodia, MD; Sanyogita Jain, MD; Sapna Katarkar, DA
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Abstract

Basaloid squamous cell carcinoma (BSCC) is rare. We report a case of BSCC in a 60-year-old woman who presented with a bleeding vascular growth on the left pinna. To the best of our knowledge, no case of BSCC of the pinna has been previously reported in the literature. We present this case to alert physicians that this highly aggressive variant of squamous cell carcinoma can appear on the pinna and therefore it should be considered in the differential diagnosis of lesions in this area.

Recurrence of a nasopharyngeal carcinoma manifesting as a cerebellopontine angle mass

December 20, 2013     Min Han Kong, MS; Jahendran Jeevanan, MS; Thanabalan Jegan, MS
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Abstract

As many as 31% of patients with nasopharyngeal carcinoma present with intracranial extension. Despite this high percentage, extension to the cerebellopontine angle is rare. The mechanism of tumor spread to the cerebellopontine angle is not completely understood. The most likely mechanism is direct extension to the skull base with involvement of the petrous apex and further extension posteriorly via the medial tentorial edge. We report the case of a 46-year-old woman with nasopharyngeal carcinoma who had been treated initially with chemoradiation and subsequently with stereotactic radiosurgery for residual tumor. One year later, she presented with an intracranial recurrence of the nasopharyngeal carcinoma in the cerebellopontine angle; the recurrence mimicked a benign tumor on magnetic resonance imaging. The tumor was ultimately diagnosed as an undifferentiated carcinoma of nasopharyngeal origin. She was treated with palliative chemotherapy.

Case report: Paraneoplastic neurologic syndrome associated with squamous cell carcinoma of the tonsil

October 23, 2013     Jeffrey R. Janus, MD; Sivakumar Chinnadurai, MD; Eric J. Moore, MD
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Abstract

Paraneoplastic syndromes include a variety of disorders that affect the neurologic, endocrine, mucocutaneous, hematologic, and other systems as a result of neoplastic disease. Although their presentations vary, syndromes occur when tumor antigens exhibit cross-reactivity to similar antigens expressed by these systems. The antigens in the nervous system are called “onconeural” antigens. Although many disorders are associated with a comparatively high incidence of paraneoplastic neurologic syndromes, only a few cases have been associated with squamous cell carcinoma (SCC) of the tonsil. We report the case of a 69-year-old man who initially presented with weakness and spastic gait. He was subsequently found to have a characteristic paraneoplastic tractopathy on thoracic magnetic resonance imaging. The subsequent workup and operative intervention identified a T2N0M0 SCC of the tonsil. Following resection, the patient's overall symptoms were significantly alleviated, and his gait improved. A thorough literature search yielded no other report of a tonsillar SCC with associated paraneoplastic thoracic spine tractopathy.

Undifferentiated thyroid carcinoma

October 23, 2013     Lester D.R. Thompson, MD
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Histologically, undifferentiated thyroid carcinomas show a variety of patterns, from sheet-like, storiform, fascicular, angiomatoid, and meningothelial to solid, exhibiting extensive lymph-vascular invasion.

Synchronous verrucous carcinoma and inverted papilloma of the lacrimal sac: Case report and clinical update

October 23, 2013     Cheryl Gustafson, MD; Eugene Einhorn, MD; Mary H. Scanlon, MD; Kenneth E. Morgenstern, MD; Paul J. Howlett, MD; Noam A. Cohen, MD, PhD
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Abstract

Inverted papilloma is a benign epithelial tumor of the nasal cavity. It is known to coexist with malignancy in 5 to 13% of cases, with squamous cell carcinoma being the most common malignancy. Another associated malignancy, one that is extremely rare, is verrucous carcinoma. To the best of our knowledge, no case of verrucous carcinoma occurring alone or in association with another neoplasm has been described in the nasolacrimal system. We report a case of synchronous verrucous carcinoma and inverted papilloma of the lacrimal sac in a 47-year-old man. The patient presented with epiphora, nasal obstruction, swelling of the left medial canthus, and drainage of a foul-smelling fluid from the left nostril. Computed tomography and magnetic resonance imaging detected the presence of a large mass occupying the left nasal cavity and sinuses with extension into the nasopharynx. In addition, bony invasion of the anteroinferomedial wall of the left orbit was noted with extension of the tumor into the orbit itself, which resulted in lateral displacement of the left medial rectus muscle. The patient underwent endoscopic debulking of the left sinonasal lesion. Of note, the surgery had to be completed in stages because of excessive blood loss. Histopathologic examination of the intranasal component of the tumor identified it as an inverted papilloma. One month after the intranasal resection, a left dacryocystectomy was performed; histopathologic examination revealed that an invasive verrucous squamous cell carcinoma had arisen within the inverted papilloma.

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