Despite the fact that bone involvement is not uncommon in some types of lymphoma, involvement of cranial vault is extremely rare. El Asri collected a systematic review of 36 articles including 38 patients with a chief complaint of subcutaneous scalp mass. There was a diversity in histologic subtype; however, DLBCL was the most frequent type [4].
Setta and coworkers reported an interesting case of primary cranial vault lymphoma, diagnosed accidentally in imaging studies following a minor head injury in a 75-year-old man. In magnetic resonance imaging, a subcutaneous frontal soft tissue mass was encountered at the level of head insult, in addition to brain parenchymal involvement without remarkable bone destruction. In tissue sampling, pathologic diagnosis was DLBCL. The tumor invasion was hypothesized to be via long vessels [5].
It is rare to see DLBCL that involves soft tissue, especially skeletal muscles, and this presentation forms less than 1% of extra nodal NHL. Hatem reported a 79-year-old man with involvement of left triceps muscle, which is extremely unusual [6].
Derenzini and colleagues performed a monocentric analysis and also reviewed data in past 2 decades. They found 16 cases of NHLs presenting as soft tissue masses treated in their center during 1996–2011 and also 67 patients were selected from 5 series and 16 case reports through a systematic review of 1990–2011 time-frame. The most common subtype was reported to be DLBCL (50% cases in both groups). The researchers reported worse prognosis in soft tissue DLBCL as opposed to soft tissue indolent B cell lymphoma and also in comparison to historical data on DLBCL patients. Regarding inferior outcome in this subtype strategies of first-line therapy, intensification was proposed [7].
On 221 patients with head and neck lymphoma (193 cases of NHL and 28 HL), Stork reported that highly aggressive NHL and HL patients were significantly younger (p < 0.0001). In this study group among NHL, 77 were indolent, 110 were aggressive, and 6 were highly aggressive. Different origins of NHL consisted of involvement of lymph nodes, tonsils, major salivary gland, sinonasal tract, and hypopharynx/larynx. Predictably, different presentations, especially in aggressive subtypes of NHL like Burkitt, and HL would make diagnostic challenges and it is recommended to perform biopsies early in diagnostic measures whenever a head and neck mass is encountered [8]
Of 56 head and neck DLBCL patients reported by Kwak, in 52%, site of origin was Waldeyer’s ring, 14% lymph nodes, 20% nasal cavities and paranasal sinuses, and 14% other sites as the submandibular gland, thyroid, and lacrimal sac [9].
A retrospective study which was conducted during 2004–2014 reported data on 110 nodal and extra nodal CD20+ DLBCL of head and neck area. Palatine tonsils were the most common extra nodal site (29.1%). Among cell origin markers (CD10, BCL6 and MUM1), MUM1was strikingly correlated with extra nodal lesions (p = 0.029). According to the results, the authors believed the extra nodal DLBCL of head and neck area may have longer survival of solely nodal cases [10].
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