Imaging of lymphomas of the head and neck Poster No.:
C-2051
Congress:
ECR 2013
Type:
Educational Exhibit
Authors:
G. M. Santandreu , E. Granell , B. Gómez-Anson , F. Nunez , J.
1
2
2
2 1
2
2
2
A. Molina , M. de Juan-Delago ; 08025, Ba/ES, Barcelona/ES Keywords:
Lymphoma, Education, MR, CT, Head and neck
DOI:
10.1594/ecr2013/C-2051
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Learning objectives To illustrate the spectrum of radiological findings and most frequent locations of head and neck lymphomas.
Background
Lymphomas are lymphoid malignancies, that present as solid tumors, differently from leukaemia, wich is a blood and bone marrow neoplasia. The first cases of lymphoma were described by Thomas Hodgkin in the year 1832. Their precise etiology remains unknown; although environmental and infectious agents have been historically implicated. Nowadays, lymphomas represent the fifth most frequent type of cancer, and the fifth leading cause of cancer death. Diagnosis of lymphoma is usually suggested by widespread lymphadenopathy. Extranodal involvement is seen not only together with widespread nodal disease, but also in the absence of any lymphadenopathy. Lymphomas are currently classified in the WHO classification as neoplastic diseases of the lymphoid tissues. Lymphomas are divided in: 1. Hodgkin lymphoma 2. No Hodgkin lymphoma: * B cell lymphoma * T cell and NK cell lymphoma Lymphoma is a disease with many faces. Like various other conditions, such as tuberculosis, lymphoma is known as a "great mimicker" and should be thus considered when a mass lesion is found anywhere in the body.
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A - Hodgkin lymphoma (HL): HL is characterised by the presence of Reed-Sternberg cells. People with a history of infectious mononucleosis are three times more likely to have the disease. The typical presention is with painless lymphadenopathy. However, around a third of people with HD, systemic symptoms manifest as fever, night sweats, weight loss, pruritus or fatigue. Hodgkin's disease is usually almost entirely confined to the lymph nodes. Extra nodal disease, although uncommon, may be found in any organ system, either as a primary manifestation or as a part of systemic dissemination. B - Non Hodgkin lymphomas (NHL): NHL are a heterogeneous group of tumours, which origins are either B or T lymphocytes. NHL represent the second fastest growing cancer in the United States. In Spain, it is estimated that about 2,500 cases of NHL are diagnosed each year, representing rates of 5.8 per 100,000 (men) and 3.8 (women). NHL is also one of the tumor groups recording a greater increase in mortality rates. In NHL, there is a higher incidence of extranodal disease and, in general, a worse prognosis than in Hodgkin disease. The WHO classification distinguishes between B cell malignancies (including follicular lymphoma and diffuse large B-cell) and malignancies of T lymphocytes and NK cells (including anaplastic large cell lymphoma). In fact, NHL comprises about 40 tumor types. CT scans are routinely used in the evaluation and follow-up of patients with all subtypes of non-Hodgkin's lymphoma. However, PET and gallium SPECT scans are more useful in aggressive subtypes (such as diffuse large B cell lymphoma), than in more indolent subtypes (such as follicular lymphoma or small lymphocytic lymphoma).
Images for this section:
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Fig. 1: Contrast CT scan of the neck. Disseminated lymphadenopathy and left parotid adenopathic mass. Diagnosis: follicular lymphoma.
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Fig. 2: Contrast CT scan of the neck (same patient as figure 1). Left parotid adenopathic mass. There are further lymphadenopathies, predominantly in the left: submandibular , digastric and internal jugular regions. Diagnosis: follicular lymphoma.
Fig. 3: Contrast CT scan of the neck. Large and necrotic lymphadenophaties located medially to the right sternocleidomastoid muscle, affecting the right sided palatine tonsillar region and growing into the lateral portion of the soft palate. Diagnosis: metastatic nodes of esophageal carcinoma. These radiological features are uncommon in lymphoma.
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Fig. 4: Contrast-enhanced CT scan of the neck. Large solid lesion involving the left amygdala, and bilateral internal jugular lymphadenopathies. Diagnosis: diffuse large cell lymphoma.
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Fig. 5: Axial postcontrast, fat suppressed MR image demonstrates an extensive tumor involving the entire nasopharynx, and displacing the soft palate. It extends laterally into the carotid space. This is the typical radiological, non-infiltrative, appearance of lymphoma. Diagnosis: diffuse large cell lymphoma.
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Fig. 6: Sagittal, postcontrast, T1-weighted MR image (same patient as figure 5) demonstrates an extensive mass involving the entire nasopharynx, and displacing the soft palate anteriorly. There are no signs of bony destruction at the skull base. This is the typical radiological, non-infiltrative, appearance of lymphoma. Diagnosis: diffuse large cell lymphoma.
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Fig. 7: PET-CT of the head and neck (acquisition 60 min after iv injection of 18F-FDG) (same patient as figures 5 and 6). Homogeneously, enhancing hypermetabolic mass. Diagnosis: diffuse large cell lymphoma.
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Fig. 8: CT scan. Right maxillary sinus occupation with associated soft tissue mass growing into the infratemporal fossa and cheek region. No bony destruction is seen. Diagnosis: diffuse large cell lymphoma.
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Fig. 9: Axial postcontrast T1-weighted MR image (same patient as figure 8). Right maxillary sinus occupation with associated soft tissue mass growing into the infratemporal fossa and cheek region. No bony destruction is seen. Diagnosis: diffuse large cell lymphoma.
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Fig. 10: Diffusion-Weighted MR Imagine demonstrates a high-signal within the tumor, related to high cellularity (same patient as figures 8 and 9): Diagnosis: diffuse large cell lymphoma.
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Fig. 11: Axial ADC map shows restricted ADCs within the tumor, related to high cellularity (same patient as in figures 8-10). Diagnosis: diffuse large cell lymphoma.Diagnosis: diffuse large cell lymphoma.
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Fig. 12: PET-CT study performed one hour after iv administration of 18F-FDG, shows increased uptake (SUVmàx 10.8) and thus high metabolism of the mass centered in the right maxillary sinus (same patient as figures 8-11). Diagnosis: diffuse large cell lymphoma.
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Fig. 13: Contrast-enhanced CT scan. Ethmoidal and maxillary sinus tumor, showing strong constrast enhancement. Bony destruction was present in the anterior cranial fossa and in the medial and inferior left orbital walls. (The patient had a pacemaker, and MR was not performed). Diagnosis: diffuse large cell lymphoma.
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Fig. 14: Helical CT scan after intravenous injection of iodinated contrast: There is a large, right intraparotid adenopathy showing contrast enhancement. Diagnosis: mixed cellularity Hodgkin lymphoma in HIV positive patient.
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Fig. 15: Helical CT scan after intravenous injection of iodinated contrast in the same patient as figure 14. Multiple enlarged lymph nodes medial to the sternocleidomastoid muscle and in the spinal chain (internal and posterior jugular chain), showing no apparent necrosis images inside are seen. Diagnosis: mixed cellularity Hodgkin lymphoma in HIV positive patient.
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Fig. 16: Contrast enhanced orbital CT. Infiltrative lesion in left orbit with solid characteristics in the periocular area. Thickening of the extraocular muscles is also present. Diagnosis: Natural killer cell lymphoma in a patient with periorbital cellulitis, and poor response to treatment.
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Fig. 17: Contrast enhanced orbital CT (same patient of the figure 16). Infiltrative solid lesion in left orbit and periocular area. Diagnosis: Natural killer cell lymphoma in a patient with periorbital cellulitis, and poor response to treatment.
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Fig. 18: CT of the neck after injection of intravenous iodinated contrast. Large thyroid mass involving the left lobe, isthmus and part of the right lobe. The mass, produces displacement of the larynx and the trachea to the right. The left jugular vein is partially collapsed and thrombosed. Diagnosis: diffuse large cell lymphoma in patient with a history of autoimmune hypothyroidism (Hashimoto's thyroiditis).
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Fig. 19: CT scan of the larynx after contrast injection (same patient as figure 18). Supraglottic mass infiltrating the epiglottis, both aryteno-epiglottic folds and the right pyriform sinus, and producing severe narrowing of the superior airway. Central necrosis was present. Diagnosis: diffuse large cell lymphoma.
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Fig. 20: CT scan of the larynx after contrast injection . Post-chemotherapy follow-up of supraglottic lymphoma, with important reduction in size(same patient as figures 18 and 19).
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Imaging findings OR Procedure details
A - General aspects: There are several statistics about head and neck lymphoma in the literature. Any area of the head or neck may be affected by lymphoma. Although Hodgkin lymphoma typically affects the cervical lymph nodes at the time of presentation and the involvement of true extranodal sites in the head and neck is rare. The presence of extranodal involvement of lymphoma in the head and neck, again, reflects the increased prevalence of NHL (seen in 25-40% of cases) over HL (seen in only 4-5% of cases). Extranodal NHL represents 5% of head and neck neoplasm. The first step in a patient with suspected lymphoma is achieving a precise histological diagnosis from biopsy. Sometimes, needle biopsy allows characterization, while samples obtained by fine needle aspiration are not usually considered valid, enough to establish a diagnosis. Once lymphoma has been confirmed, or is strongly suspected clinically, then wholebody CT (neck, chest, and abdomen and pelvis) with IV contrast material is the first investigation of choice. The accuracy of MRI to detect nodal involvement is equal to that of CT and offers no advantage. However, Magnetic resonance imaging is useful in delineating the extent of the tumor and adjacent organ involvement. Diffusion-weighted imaging is potentially useful in the evaluation of head and neck lesions and could be used to distinguish lymphomas from squamous cell carcinomas (SCC), as ADC values of lymphomas tend to be significantly lower than those of SCC. The most simple and cost-effective method probably is the US. Nevertheless, the utility of US is for confirmation of nodal involvement in a palpable mass. PET has now become part of the routine diagnostic workup of oncologic patients, particularly those with highgrade tumors, as these are more likely to show avid 18 F-FDG uptake. There is a Known limitation of CT to distinguishing viable residual tumor from masses containing only fibrosis or necrosis. PET may detect the existence of viable tumor, but there are false negatives (low tumor burden) as well as false positives (inflammatory and
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infectious causes, rebound thymic changes post radiotherapy, bone marrow hyperplasia secondary treatment with growth factors, etc.). Faced with a positive PET-scan after finishing treatment, and since its positive predictive value is not 100%, a potential relapse must be confirmed with biopsy. The goal of treatment is to achieve complete remission. Evaluation of the response to treatment is usually performed with CT and/or PET.
B - Lymph node involvement: Widespread lymphadenophathy is the most common presentation of lymphomas (Fig. 1 on page 29, Fig. 2 on page 30). The main problem with lymphadenopathy is differentiating between lymphoma and involvement by other inflammatory or tumoral conditions. Lymphoma remains second in frequency to squamous cell carcinoma (SCC) in head and neck cancers; and both may present with widespread nodal and extranodal disease (Fig. 3 on page 32). The presence of necrosis within lymph nodes and the absence of distant nodal or extranodal disease suggests SCC rather than lymphoproliferative disease. However, necrosis may be present in 5% of enlarged nodes by lymphoma. Similarly, the presence of skull base erosion in a lesion arising from the nasopharyngeal wall should suggest SCC. Nevertheless, in som cases, imaging of lesions in this region may be inconclusive, and biopsy may be required for formal diagnosis. Inflammatory processes may also result in lymphadenopathy; having the same radiological appearance. This is the case for both acute (abscesses or mononucleosis) and chronic process (like tuberculosis), and for in less common diseases like toxoplasmosis. Of course, the final diagnosis can not be made purely based on imaging, and needs to consider the clinical context of the patient, as well as physical examination.
C - Involvement of Waldeyer ring, pharynx and oral cavity:
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Waldeyer ring is a ring of lymphoid tissue located in the nasopharynx and oropharynx at the entrance of the aerodigestive tract. The structures composing this ring are the palatine tonsils, the adenoids (nasopharyngeal tonsils), the lateral walls of the oropharynx and the lingual tonsil at the base of the tongue. Lymphoma may involve the pharynx primarily or result from an extension from head and neck disease. This is the most common site for extranodal involvement by diffuse large B-cell lymphoma (Fig. 4 on page 32 ). Pharyngeal nodes and soft-tissue masses due to lymphoma may be difficult to differentiate from SCC involving the tongue or palatine fossa Nasopharyngeal lymphoma is the second most common malignancy in the adult patients after squamous cell carcinoma. In most cases corresponds to non-Hodgkin lymphomas, which develop primarily in the Waldeyer ring. The little infiltrative nature of this tumor may, sometimes, make it difficult to be differentiated from simple hypertrophy of pharyngeal adenoid tissue. Diagnosis of lymphoma should be suspected when extranodal tumor areas, or diffuse involvement of Waldeyer's ring are also present. Radiologically, it is difficult to distinguish from SCC, but generally nasopharyngeal lymphoma is a more expansive tumor and less infiltrative, is associated with large lymphadenophaties without central necrosis, which do not follow typical lymphatic drainage pathways. The diagnosis of nasopharyngeal lymphoma should be suspected when simultaneous extranodal foci tumor are detected (sinus, orbit, space chewing, jaw, salivary gland, skin, and larynx), or there is diffuse involvement of Waldeyer's ring (Fig. 5 on page 33 , Fig. 6 on page 34, Fig. 7 on page 35). Tongue lesions may appear as subtle alterations in the muscle texture on MRI, and the presence of an associated mass effect may help in identifying lesions. Unlike malignant lymphoma of Waldeyer's ring, malignant lymphoma of the oral cavity is rare and almost exclusively of the NHL type. It is more common to AIDS patients. Burkitt's lymphoma is seen as a rapidly expanding mass involving the mandible and is particularly associated with Epstein-Barr virus infection.
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D - Sinonasal involvement: Sinonasal lymphomatous masses may be large and destructive, extending to the orbit. They are difficult to be differentiated from other pathologic entities, typically ocurrying at this site, such us Wegener granulomatosis, SCC, or esthesioneuroblastoma. However, lymphoma tends to arise in the nasal cavity, whereas SCC tends to start out in the paranasal sinuses. CT scan is useful in assessment of bone integrity. Tumor extension through sinusal wall (or any other bone structure), without bony destruction is an almost pathognomonic feature. MRI characteristics may also be useful in distinguishing among these entities. Lymphoma tends to be more heterogeneous on T2-weighted sequences, having moderately intense, homogeneous enhancement (Fig. 8 on page 36, Fig. 9 on page 37, Fig. 10 on page 38, Fig. 11 on page 39, Fig. 12 on page 40, Fig. 13 on page 41).
E - Salivary glands involvement: Unlike other salivary gland malignancies, lymphoma is more common in the parotid gland than in the submandibular or sublingual glands and is typically bilateral. Most of the times, it presents as widespread nodal disease. Bronwyn et al. published a review of the imaging and clinical features of parotid tail masses in 103 patients (the parotid tail is the most inferior portion of the superficial lobe). Parotid lymphoma was the lesion most likely to present with multifocal and/or bilateral masses and was the most common malignancy in the parotid tail. This is inconsistent with results of prior series, which suggest that both primary and secondary parotid lymphoma are relatively uncommon(Fig. 14 on page 42, Fig. 15 on page 43). F - Orbital involvement: Orbital lymphomas constitute 5-15% of all extranodal lymphomas, and about 50% of all primary orbital malignancies in adults. The possible infective etiology may explain the observed increase in porevalence of orbital lymphomas. Orbital lymphoma is, most frequently, a B-cell NHL, and in most cases arises from mucosa-associated lymphoid tissue. Masses may cause painless gradual orbital proptosis with preservation of vision.
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Orbital lymphoma usually appears as a soft tissue mass, either involving the conjunctiva or elsewhere in the orbit. Masses are more frequent in the upper outer quadrant, and involve the lacrimal gland, this being bilateral in up to 25% of cases. Although the extraocular muscles may be surrounded or displaced by the mass, they can usually be identified as not being the origin of the tumour, a sign which results helpful in distinguishing lymphomas from other orbital masses. Invasion of the globe or the optic nerve is rare. On contrast CT, the mass is usually homogeneous in density, being either isodense or slightly hyperdense when compared to the extraocular muscles (Fig. 16 on page 44, Fig. 17 on page 45). On MRI orbital lymphoma usually shows an intermediate-signal on T1- and T2-weighted imaging, and there is moderate enhancement .
G - Thyroid involvement: Primary thyroid lymphoma is a rare disease, that results in diagnostic and therapeutic dilemmas. They are uncommon tumours, representing only 5% of the thyroid neoplasms. These B-cell NHL are associated in 80% of the cases with a previous Hashimoto thyroiditis. Imaging techniques are unable to differentiate between thyroiditis and primary thyroid lymphoma. On ultrasound, CT and MRI, lymphoma is seen as a homogeneous mass without necrosis or calcifications. After contrast administration, there is absence of enhancement. These tumors are hypoechoic in US, and hypodense on CT. Fine needle aspiration of thyroid lesions may prove difficult to provide a diagnosis of lymphoma, particularly if there is a background lymphocytic infiltrate associated to Hashimoto disease (Fig. 18 on page 46).
H - Laryngeal involvement:
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Primary NHL of the larynx is a rare entity accounting for 1% of laryngeal tumors. Laryngeal lymphoma is an important differential consideration because lymphoma is treated with chemoradiation instead of surgery. NHL is presumed to arise from 2 locations within the larynx: aggregates of specialized lymphoid tissue in the submucosa, predominantly of B-cell lineage, or MALT, predominantly arising from the aryepiglottic folds and epiglottis. The symptons are site related and non-specific, being undistinguished from other neoplasms. A large, non-necrotic, supraglottic lesion with a submucosal component that demonstrates homogeneous enhancement is more characteristic of lymphoma than of squamous cell carcinoma. Laryngeal lymphoma is commonly seen to extend into the hypopharynx, but it can also ocur just at thesupraglottis (Fig. 19 on page 47, Fig. 20 on page 48). Calcifications are not seen with laryngeal lymphoma. Like squamous cell carcinoma, lymphoma can extend into the subglottis, oropharynx, strap muscles, and laryngeal cartilage. Images for this section:
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Fig. 1: Contrast CT scan of the neck. Disseminated lymphadenopathy and left parotid adenopathic mass. Diagnosis: follicular lymphoma.
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Fig. 2: Contrast CT scan of the neck (same patient as figure 1). Left parotid adenopathic mass. There are further lymphadenopathies, predominantly in the left: submandibular , digastric and internal jugular regions. Diagnosis: follicular lymphoma.
Fig. 3: Contrast CT scan of the neck. Large and necrotic lymphadenophaties located medially to the right sternocleidomastoid muscle, affecting the right sided palatine tonsillar region and growing into the lateral portion of the soft palate. Diagnosis: metastatic nodes of esophageal carcinoma. These radiological features are uncommon in lymphoma.
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Fig. 4: Contrast-enhanced CT scan of the neck. Large solid lesion involving the left amygdala, and bilateral internal jugular lymphadenopathies. Diagnosis: diffuse large cell lymphoma.
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Fig. 5: Axial postcontrast, fat suppressed MR image demonstrates an extensive tumor involving the entire nasopharynx, and displacing the soft palate. It extends laterally into the carotid space. This is the typical radiological, non-infiltrative, appearance of lymphoma. Diagnosis: diffuse large cell lymphoma.
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Fig. 6: Sagittal, postcontrast, T1-weighted MR image (same patient as figure 5) demonstrates an extensive mass involving the entire nasopharynx, and displacing the soft palate anteriorly. There are no signs of bony destruction at the skull base. This is the typical radiological, non-infiltrative, appearance of lymphoma. Diagnosis: diffuse large cell lymphoma.
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Fig. 7: PET-CT of the head and neck (acquisition 60 min after iv injection of 18F-FDG) (same patient as figures 5 and 6). Homogeneously, enhancing hypermetabolic mass. Diagnosis: diffuse large cell lymphoma.
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Fig. 8: CT scan. Right maxillary sinus occupation with associated soft tissue mass growing into the infratemporal fossa and cheek region. No bony destruction is seen. Diagnosis: diffuse large cell lymphoma.
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Fig. 9: Axial postcontrast T1-weighted MR image (same patient as figure 8). Right maxillary sinus occupation with associated soft tissue mass growing into the infratemporal fossa and cheek region. No bony destruction is seen. Diagnosis: diffuse large cell lymphoma.
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Fig. 10: Diffusion-Weighted MR Imagine demonstrates a high-signal within the tumor, related to high cellularity (same patient as figures 8 and 9): Diagnosis: diffuse large cell lymphoma.
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Fig. 11: Axial ADC map shows restricted ADCs within the tumor, related to high cellularity (same patient as in figures 8-10). Diagnosis: diffuse large cell lymphoma.Diagnosis: diffuse large cell lymphoma.
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Fig. 12: PET-CT study performed one hour after iv administration of 18F-FDG, shows increased uptake (SUVmàx 10.8) and thus high metabolism of the mass centered in the right maxillary sinus (same patient as figures 8-11). Diagnosis: diffuse large cell lymphoma.
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Fig. 13: Contrast-enhanced CT scan. Ethmoidal and maxillary sinus tumor, showing strong constrast enhancement. Bony destruction was present in the anterior cranial fossa and in the medial and inferior left orbital walls. (The patient had a pacemaker, and MR was not performed). Diagnosis: diffuse large cell lymphoma.
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Fig. 14: Helical CT scan after intravenous injection of iodinated contrast: There is a large, right intraparotid adenopathy showing contrast enhancement. Diagnosis: mixed cellularity Hodgkin lymphoma in HIV positive patient.
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Fig. 15: Helical CT scan after intravenous injection of iodinated contrast in the same patient as figure 14. Multiple enlarged lymph nodes medial to the sternocleidomastoid muscle and in the spinal chain (internal and posterior jugular chain), showing no apparent necrosis images inside are seen. Diagnosis: mixed cellularity Hodgkin lymphoma in HIV positive patient.
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Fig. 16: Contrast enhanced orbital CT. Infiltrative lesion in left orbit with solid characteristics in the periocular area. Thickening of the extraocular muscles is also present. Diagnosis: Natural killer cell lymphoma in a patient with periorbital cellulitis, and poor response to treatment.
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Fig. 17: Contrast enhanced orbital CT (same patient of the figure 16). Infiltrative solid lesion in left orbit and periocular area. Diagnosis: Natural killer cell lymphoma in a patient with periorbital cellulitis, and poor response to treatment.
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Fig. 18: CT of the neck after injection of intravenous iodinated contrast. Large thyroid mass involving the left lobe, isthmus and part of the right lobe. The mass, produces displacement of the larynx and the trachea to the right. The left jugular vein is partially collapsed and thrombosed. Diagnosis: diffuse large cell lymphoma in patient with a history of autoimmune hypothyroidism (Hashimoto's thyroiditis).
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Fig. 19: CT scan of the larynx after contrast injection (same patient as figure 18). Supraglottic mass infiltrating the epiglottis, both aryteno-epiglottic folds and the right pyriform sinus, and producing severe narrowing of the superior airway. Central necrosis was present. Diagnosis: diffuse large cell lymphoma.
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Fig. 20: CT scan of the larynx after contrast injection . Post-chemotherapy follow-up of supraglottic lymphoma, with important reduction in size(same patient as figures 18 and 19).
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Conclusion Lymphoma must be included in the differential diagnosis of extracranial head and neck masses. CT and MR imaging may provide strong clues to suggest the diagnosis of lymphoma, allowing its differentiation from other entities, such as SCC. The role of cross-sectional CT and MRI techniques, includes diagnosis, staging and monitoring disease progression or treatment response.
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según las principales localizaciones tumorales, 1985-2019:linfomas no Hodgkin. Med Clin (Barc). 2008;131(Supl 1):72-77. 9. Paes, F. et al. FDG PET/CT of Extranodal Involvement in Non-Hodgkin Lymphoma and Hodgkin Disease . Radiographics January 2010 30:1 269-291. 10. Rovira, A. ; De Juan, M. ; Ramos, A. Radiología de Cabeza y Cuello. SERAM. Editorial Médica Panamericana 2009, 23-35; 69-81. 11. Siddiqui, N. et al. Imaging Characteristics of Primary Laryngeal Lymphoma. AJNR 2010 31: 1261-1265. Page 50 of 51
12. Sumi, M. Discrimination of Metastatic Cervical Lymph Nodes with DiffusionWeighted MR Imaging in Patients with Head and Neck Cancer. AJNR 2003 24:1627-1634. 13. Thomas, A. et al. Extranodal Lymphoma From Head to Toe: Part 1, The Head and Spine. AJR 2011 197:350-356. 14. Weber, A. L. et al. Hodgkin and non-Hodgkin lymphoma of the head and neck: clinical, pathologic, and imaging evaluation. Neuroimaging Clin N Am 2003 Aug;13(3):371-92.
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