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Hodgkin lymphoma

Hodgkin's lymphoma, formerly known as Hodgkin's disease, is a type of lymphoma described by Thomas Hodgkin in 1832, and characterized by the presence of Reed-Sternberg cells. more...

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Unlike other lymphomas, whose incidence increases with age, Hodgkin's lymphoma has a bimodal incidence curve: that is, it occurs more frequently in two separate age groups, the first being young adulthood (age 15-35), the second being in those over 50 years old. Overall, it is more common in males, except for the nodular sclerosis variant (see below) of Hodgkin disease, which is more common in women.

The incidence of Hodgkin's disease is about 4/100,000 people/year, and accounts for a bit less than 1% of all cancers worldwide.


Swollen, but non-painful, lymph nodes are the most common sign of Hodgkin's lymphoma, often occurring in the neck. The lymph nodes of the chest are often affected and these may be noticed on a chest X-ray.

Splenomegaly, or enlargement of the spleen, occurs in about 30% of people with Hodgkin's lymphoma. The enlargement, however, is seldom massive. The liver may also be enlarged due to liver involvment in the disease in about 5% of cases.

About one-third of people with Hodgkin's disease may also notice some systemic symptoms, such as low-grade fever, night sweats, weight loss, itchy skin (pruritis), or fatigue. Systemic symptoms such as fever and weight loss are known as B symptoms.


Hodgkin's lymphoma must be distinguished from non-cancerous causes of lymph node swelling (such as various infections) and from other types of cancer. Definitive diagnosis is by lymph node biopsy (removal of a lymph node for pathological examination). Blood tests are also performed to assess function of major organs, to detect lymphoma deposits or to assess safety for chemotherapy. Positron emission tomography is used to detect small deposits that do not show on CT scanning.



Affected lymph nodes (most often, laterocervical lymph nodes) are enlarged, but their shape is preserved because the capsule is not invaded. Usually, the cut surface is white-grey and uniform; in some histological subtypes (e.g. nodular sclerosis) may appear a nodular aspect.


Microscopic examination of the lymph node biopsy reveals complete or partial effacement of the lymph node architecture by scattered large malignant cells known as Reed-Sternberg cells (typical and variants) admixed within a reactive cell infiltrate composed of variable proportions of lymphocytes, histiocytes, eosinophils, and plasma cells. The Reed-Sternberg cells are identified as large often binucleated cells with prominent nucleoli and an unusual CD45-, CD30+, CD15+/- immunophenotype. In approximately 50% of cases, the Reed-Sternberg cells are infected by the Epstein-Barr virus.


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Primary non-Hodgkin's lymphoma of the frontal sinus: How we diagnosed it
From Ear, Nose & Throat Journal, 9/1/00 by Hamdy El-Hakim


Isolated primary non-Hodgkin's lymphoma of the frontal sinus is rare. We describe the case of a middle-aged man who came to us with signs of orbital cellulitis complicating an acute infected frontal mucocele. His condition was initially controlled with medical therapy and subsequent endoscopic sinus surgery, but his symptoms eventually returned. We were able to diagnose the lymphoma only by approaching the sinus externally to obtain a biopsy. This case highlights the importance of making a full visual inspection of the involved sinus in order to avoid missing an unexpected, albeit a rarely encountered, pathology.


Isolated non-Hodgkin's lymphoma of the frontal sinus is a rare diagnosis that has been seldom documented in the literature. It is usually widespread and involves the other sinuses and/or the nasal cavity. [1] On the other hand, the frontal and the ethmoid sinuses are common sites of mucocele development Mucoceles are increasingly being managed endoscopically, with or without the concomitant use of an external approach. [2] In this article, we report a case of lymphoma that was diagnosed only after we used a traditional external procedure.

Case report

A 58-year-old white man came to the otolaryngology department with a 48-hour history of left medial canthus edema and erythema, which had eventually spread across the entire periorbital region. He also complained of bilateral frontal headaches, anterior and posterior rhinorrhea, and intermittent but brisk epistaxis. He was aware of a nasal obstruction, and he had noticed a gradual diminution of his sense of smell. He also said he had noticed the development of a hard, bony swelling at the left medial canthus during the previous 6 months. The man smoked 20 cigarettes daily and drank alcohol only socially. His history was significant for asthma, hypertension, a head injury, and chronic rhinosinusitis, for which he had undergone an antral washout when he was 13 years old.

On admission, the patient was apyrexial and not systemically ill. The periorbital swelling, erythema, and tenderness were more marked over the medial canthus and the frontal sinus on the left. There was no limitation of eye movements or visual acuity. Nasoendoscopy under topical anesthesia revealed a mild deviation of the nasal septum and bilateral edematous middle and inferior turbinates and middle meatal polyps. We also noted the presence of mucopurulent rhinorrhea.

Urgent computed tomography (CT) revealed the presence of bilateral mucosal disease in the ethmoid, maxillary, and frontal sinuses--the latter being totally opacified (figure 1). There was a loss of definition of the bony floor of the left frontal sinus and an apparent bony breach. The patient's complete blood count (CBC) did not reveal any specific abnormality (hemoglobin: 144 g/dl; platelets: 319,000/[micro]l; white cells: 6,700/[micro]l).

The patient was diagnosed as having an infected left frontal sinus mucocele. He was started on intravenous metronidazole (500 mg tid), IV ampicillin/flucloxacillin (1 g qid), and topical xylometazoline nasal drops (2 drops tid). The soft tissue swelling resolved over the next 48 hours, and the IV medications were replaced with oral metronidazole (400 mg tid) and amoxicillin/clavulanate (375 mg tid).

Seven days later, the patient underwent an endoscopic bilateral uncinectomy, a middle meatal antrostomy, and an anterior ethmoidectomy. Polyps blocking the frontal recess opening into the middle meatus were cleared, agger nasi cells were exenterated, and mucus was drained from the frontal sinus. All removed tissues were sent for histopathologic examination. The oral antibiotics and topical decongestant drops were continued for 10 days postoperatively. There were no operative complications, and the patient was instructed to begin using topical fluticasone spray (100 [micro]g in each nostril once a day) in 10 days and to continue taking it for 2 months.

Over the next 4 months, the bony swelling resolved and the patient's symptoms improved. The antrostomies began to gradually narrow, and elective revision surgery was planned. Histology of the removed tissues revealed inflammation but no evidence of malignancy.

Two months later, the patient returned and complained of the same symptoms he had originally reported. The swelling had spread to the contralateral medial canthus, and he occasionally experienced double vision. The clinical findings were identical to those seen on the previous occasion. In addition, the tender red swelling extended from the left medial canthus over the glabella to the right medial canthus. The swelling had displaced the left eye inferiorly, which caused diplopia in the extreme upward gaze. Visual acuity and color vision were not affected. Nasoendoscopic findings were similar to those seen earlier.

Another urgent CT showed, in addition to the bony erosion of the left frontal sinus floor, an extension of a soft tissue mass from the sinus into the soft tissues of the upper eyelid and the forehead (figure 2). The mass was eroding the intersinus septum and the posterior wall of the sinus. CBC results were again all within normal ranges. The original drug regimen with IV antibiotics and topical decongestants was reinstated.

On this occasion, it was decided to approach the frontal sinus externally. After a Lynch-Howarth incision was made, a 2-cm dehiscence was found on the floor of the left frontal sinus. Through the dehiscence, an irregular soft tissue mass appeared to have pushed, but did not infiltrate, the soft tissues of the upper eyelid. The mass extended to the contralateral frontal sinus and breached the posterior bony wall, but it did not infiltrate the dura. Purulent material was encountered during dissection of the mass from the bony margin. The frontal recess was clear, the ethmoid cavity was healthy, and there was no evidence of osteomyelitis. The mass was excised, and the wound was closed in two layers. Three doses of IV amoxicillin/clavulanate (1.2 g) were administered postoperatively, and then the patient was prescribed an oral course (375 mg tid) for 2 weeks.

A histopathologic diagnosis of high-grade B-cell non-Hodgkin's lymphoma was made. There was no evidence of extension into any other sinus. CBC and biochemistry, whole-body CT, lumbar puncture, bone marrow aspirate, and trephine biopsy findings were all normal. The disease was designated as stage 1E.

The patient received six courses of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP). In view of the high risk of central nervous system involvement, a lumbar puncture was performed, which revealed no evidence of lymphoma. Intrathecal methotrexate was prescribed after each course of CHOP.

Three months after therapy, some residual periorbital edema had persisted, but there was no clinical or radiologic evidence of disease progression.


In the Western population, lymphomas of the nasal cavity and paranasal sinuses rank among the uncommon extranodal lymphomas. They account for only 0.17% of all lymphomas and 0.43% of all extranodal lymphomas in the Kiel Lymph Node Registry. [3] Relative to other malignant neoplasms of the paranasal sinuses, their incidence is again low; for example, they accounted for only 8% of all sinus tumors discovered at the Massachusetts Eye and Ear Infirmary between 1946 and 1970. [4] The clinical presentation of these lesions can be confused with those of infectious, granulomatous, and nonlymphomatous neoplastic processes. [5] The limited clinical experience with this lesion has led to controversy regarding its pathologic classification, natural history, and optimal management. [1]

The most common primary sites are the maxillary antrum, the nasal cavity, and the ethmoids. Most lesions are locally advanced and involve multiple anatomic structures. This makes the assignment of a primary site somewhat arbitrary in cases of large tumors.

In one of the largest studies of this type of lymphoma, comprising 70 patients who were treated between 1947 and 1993, Logsdon et al reported a high male predominance (73%) and a mean age of 57 years. [1]

Our patient's clinical picture suggested the presence of an acute orbital cellulitis complicating an infected frontal sinus mucocele. A divide has existed between Great Britain and the United States with regard to the treatment of frontoethmoid mucoceles. In general, the osteoplastic flap approach remains the most popular definitive method in the United States, whereas many British surgeons favor the Lynch-Howarth frontoethmoidectomy. [6]

In 1989, Kennedy et al demonstrated the potential of treating mucoceles via the endoscopic route in order to dramatically reduce morbidity while maintaining a comparable success rate. [7] In 1998, their findings were substantiated by Lund, who reported a series of 48 cases. [2] Twenty of these patients had been managed successfully with an endoscopic approach alone, while the other 28 needed a combined approach.

Our patient's CT evaluation demonstrated that the lesion was not confined to the lateral or superior portion of the frontal sinus; nor was it limited to the internal os of the frontal recess (in fact, it actually extended through it). Moreover, CT ruled Out orbital invasion and intracranial extension which, according to Kennedy et al, [7] are criteria for endoscopic marsupialization.

Despite the success in draining a mucocele of the frontal sinus, the malignant lesion was missed because the sinus cavity could not be inspected in its entirety with angled endoscopes. Moreover, the diseased tissue that was accessible in the ethmoids and the frontal recess was not histopathologically malignant. The external approach, which was employed later, afforded superior visualization, which aided in diagnosis and management.

From the Department of Otolaryngology, Aberdeen Royal Infirmary, Aberdeen, Scotland.


(1.) Logsdon MD, Ha CS. Kavadi VS, et al. Lymphoma of the nasal cavity and paranasal sinuses: Improved outcome and altered prognostic factors with combined modality therapy. Cancer 1997;80:477-88.

(2.) Lund VJ. Endoscopic management of paranasal sinus mucocoeles, J Laryngol Otol 1998;112:36-40.

(3.) Fellbaum C, Hansmann ML, Lennert K. Malignant lymphomas of the nasal cavity and paranasal sinuses. Virchows Arch A Pathol Anat Histopathol 1989;414:399-405.

(4.) Sofferman RA, Cummings CW. Malignant lymphoma of the paranasal sinuses. Arch Otolaryngol 1975;101:287-92.

(5.) Wilder WH, Harner SG. Banks PM. Lymphoma of the nose and paranasal sinuses. Arch Otolaryngol 1983;109:310-2.

(6.) Rubin JS, Lund VJ, Salmon B. Frontoethmoidectomy in the treatment of mucoceles. A neglected operation. Arch Otolaryngol Head Neck Surg 1986;112:434-6.

(7.) Kennedy DW, Josephson JS, Zinreich SJ, et al. Endoscopic sinus surgery for mucoceles: A viable alternative. Laryngoscope 1989;99:885-95.

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