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primary adenocarcinoma of the orbit: a case study

primary adenocarcinoma of the orbit: a case study

Introduction

Primary mucinous adenocarcinoma is an anomalous neoplasm with a tendency to frequently recur and metastasize. It is a rare neoplasm with no predisposing factors or etiology. It can often be seen throughout the body, especially in the head or neck. Symptoms include abdominal pain and/or blood in the stool but can also include fatigue, weight loss, and changes in bowel habits. Treatment typically involves surgical removal of the affected area, followed by chemotherapy and/or radiation therapy to kill any remaining cancer cells.

Mucinous adenocarcinomas that arise from the skin have always had a tendency to grow near the eyelid. In fact, the rate of occurrence of this tumor in the eyelid varied from 30 to 45% according to several studies. The average age of patients with primary mucinous adenocarcinoma varied between the sixth and seventh decades. 

Mucinous adenocarcinomas of the skin will generally need to be surgically removed, and additional treatments such as chemotherapy or radiation may be necessary. Depending on the size and stage of cancer, immunotherapy or targeted therapies may be suggested. Basal cell tumors are the most common type of malignant eyelid tumor, making up approximately 8090% of all cases.

Primary mucinous adenocarcinoma is an uncommon type of neoplasm with a propensity for local recurrence and metastasis. Studies reveal that its origin might be in the breast, thyroid, or gastrointestinal tract. 

Although eyelid neoplasm is common in ophthalmology literature, the intraconic localization isn’t described. There’s also only one case of infiltrating adenocarcinoma with some features suggestive of mucoepidermoid carcinoma of the orbit that has been reported in the literature.

Case Report

A 66-year-old man without serious medical history was admitted to the neurosurgery department suffering from pain, progressive protrusion of the left eye, and a deep drop in vision on the left for several weeks. There is neither diplopia nor periorbital swelling. There were no other systemic complaints. 

The patient’s first external examination revealed significant proptosis with downward displacement of the left globe with no signs of lagophthalmos. A limitation of abduction was also noted. Visual acuity without correction was normal in the right eye whereas in the left one there was no perception of light. No papillary edema was revealed. Intraocular pressures were 18 mm Hg on the right and 16 mm Hg in the left. His blood tests were normal.

The patient was operated on via a combined approach, and complete enucleation was done. The final pathologic diagnosis was mucinous adenocarcinoma of the orbit. The postoperative neuroimaging showed complete resection of the tumor. The patient is referred for adjuvant radiotherapy. A CT of the orbit was made 3 months postoperatively and did not show any local recurrence.

Discussion

The patient in this study was 66 years old, which is compatible with the previous studies. The problem here is to distinguish whether it is a primary mucinous adenocarcinoma of the orbit or is it just an orbital metastasis of a known adenocarcinoma. Further study is always encouraged to determine the origin of this neoplasm.

It is also worth noting that metastatic lesions involving the orbit were found to be rare, comprising between 1% and 13% of all orbital tumors. Prompt surgical intervention with biopsy enabled a definitive diagnosis of mucinous adenocarcinoma, leading to the discovery of the primitive tumor. So far, there have been only 6 known cases of metastatic mucinous adenocarcinoma in the eye orbit. 

Five arose from gastrointestinal primaries including rectal, esophageal, gastric, and pancreatic cancer. Meanwhile, the sixth represented orbital reoccurrence from a primary eyelid sweat gland mucinous adenocarcinoma. At times, ocular symptoms have preceded the diagnosis of the primary lesion. And this highlights the importance of a thorough investigation aiming to search for the primary neoplastic process.

In medical literature, there is only one case of primary infiltrating adenocarcinoma of the orbit, with some features suggestive of carcinoma initially diagnosed as idiopathic sclerosing orbital inflammation. The tumor did not appear to be cytologically aggressive but rather had an infiltrative growth pattern suggestive of a high-grade tumor, T4N0M0.

As demonstrated in that case, even though the biopsy concluded in benign lesion, the clinician must remain vigilant if he suspects signs of malignancy. This is particularly true in the presence of atypical presentation or signs of severity. 

There are no radiological specificities in this type of tumor compared with other orbital masses. The MRI of the orbit with and without contrast may show a heterogenous orbital process, well limited, or infiltrating, with an important enhancement after injection. This lesion can be both intra-and extra-conal and can exert a mass effect on the rectus muscles and the optic nerve. 

Regardless of the orbital mass nature, clinically these tumors usually present symptoms such as proptosis, which is due to the infiltration of fat and extra-ocular muscles by the tumor. Muscle involvement has been noted to produce diplopia. The pain was generally associated with periosteal and bone involvement. Pulsations were due to bone destruction or vascular tumors. Ptosis, a palpable mass, enophthalmos, and decreased vision had also been also observed.

Conclusion 

Primary mucinous adenocarcinoma of the orbit is one of the rarest types of neoplasm. This case is said to be the second case described in the literature. Primary mucinous adenocarcinoma is a rare form of cancer because it is usually found in the appendix, which is a small organ, and it is also a rare form of adenocarcinoma. It is also difficult to diagnose because the symptoms that it causes are often vague and subtle.

A few cases of metastatic mucinous adenocarcinoma in the orbit have been described. It is worthwhile noting that histological and immunohistochemical features have been extremely helpful in the 1diagnosis of the primary etiology, but they surely could not exclude the metastatic etiology. 

Primary mucinous adenocarcinoma is locally aggressive with a recurrence rate of up to 40%, which is why the mainstay of its treatment is a large local excision. The formerly cited entities have been extremely difficult to diagnose. A workup must be done to look for a primary lesion in all patients with identified mucinous adenocarcinoma.

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